emphysema case study pdf

• LOGIN / FREE TRIAL

‘Racism absolutely must not be tolerated’

STEVE FORD, EDITOR

• You are here: COPD

Diagnosis and management of COPD: a case study

04 May, 2020

This case study explains the symptoms, causes, pathophysiology, diagnosis and management of chronic obstructive pulmonary disease

This article uses a case study to discuss the symptoms, causes and management of chronic obstructive pulmonary disease, describing the patient’s associated pathophysiology. Diagnosis involves spirometry testing to measure the volume of air that can be exhaled; it is often performed after administering a short-acting beta-agonist. Management of chronic obstructive pulmonary disease involves lifestyle interventions – vaccinations, smoking cessation and pulmonary rehabilitation – pharmacological interventions and self-management.

Citation: Price D, Williams N (2020) Diagnosis and management of COPD: a case study. Nursing Times [online]; 116: 6, 36-38.

Authors: Debbie Price is lead practice nurse, Llandrindod Wells Medical Practice; Nikki Williams is associate professor of respiratory and sleep physiology, Swansea University.

• This article has been double-blind peer reviewed
• Scroll down to read the article or download a print-friendly PDF here (if the PDF fails to fully download please try again using a different browser)

Introduction

The term chronic obstructive pulmonary disease (COPD) is used to describe a number of conditions, including chronic bronchitis and emphysema. Although common, preventable and treatable, COPD was projected to become the third leading cause of death globally by 2020 (Lozano et al, 2012). In the UK in 2012, approximately 30,000 people died of COPD – 5.3% of the total number of deaths. By 2016, information published by the World Health Organization indicated that Lozano et al (2012)’s projection had already come true.

People with COPD experience persistent respiratory symptoms and airflow limitation that can be due to airway or alveolar abnormalities, caused by significant exposure to noxious particles or gases, commonly from tobacco smoking. The projected level of disease burden poses a major public-health challenge and primary care nurses can be pivotal in the early identification, assessment and management of COPD (Hooper et al, 2012).

Grace Parker (the patient’s name has been changed) attends a nurse-led COPD clinic for routine reviews. A widowed, 60-year-old, retired post office clerk, her main complaint is breathlessness after moderate exertion. She scored 3 on the modified Medical Research Council (mMRC) scale (Fletcher et al, 1959), indicating she is unable to walk more than 100 yards without stopping due to breathlessness. Ms Parker also has a cough that produces yellow sputum (particularly in the mornings) and an intermittent wheeze. Her symptoms have worsened over the last six months. She feels anxious leaving the house alone because of her breathlessness and reduced exercise tolerance, and scored 26 on the COPD Assessment Test (CAT, catestonline.org), indicating a high level of impact.

Ms Parker smokes 10 cigarettes a day and has a pack-year score of 29. She has not experienced any haemoptysis (coughing up blood) or chest pain, and her weight is stable; a body mass index of 40kg/m 2 means she is classified as obese. She has had three exacerbations of COPD in the previous 12 months, each managed in the community with antibiotics, steroids and salbutamol.

Ms Parker was diagnosed with COPD five years ago. Using Epstein et al’s (2008) guidelines, a nurse took a history from her, which provided 80% of the information needed for a COPD diagnosis; it was then confirmed following spirometry testing as per National Institute for Health and Care Excellence (2018) guidance.

The nurse used the Calgary-Cambridge consultation model, as it combines the pathological description of COPD with the patient’s subjective experience of the illness (Silverman et al, 2013). Effective communication skills are essential in building a trusting therapeutic relationship, as the quality of the relationship between Ms Parker and the nurse will have a direct impact on the effectiveness of clinical outcomes (Fawcett and Rhynas, 2012).

In a national clinical audit report, Baxter et al (2016) identified inaccurate history taking and inadequately performed spirometry as important factors in the inaccurate diagnosis of COPD on general practice COPD registers; only 52.1% of patients included in the report had received quality-assured spirometry.

Pathophysiology of COPD

Knowing the pathophysiology of COPD allowed the nurse to recognise and understand the physical symptoms and provide effective care (Mitchell, 2015). Continued exposure to tobacco smoke is the likely cause of the damage to Ms Parker’s small airways, causing her cough and increased sputum production. She could also have chronic inflammation, resulting in airway smooth-muscle contraction, sluggish ciliary movement, hypertrophy and hyperplasia of mucus-secreting goblet cells, as well as release of inflammatory mediators (Mitchell, 2015).

Ms Parker may also have emphysema, which leads to damaged parenchyma (alveoli and structures involved in gas exchange) and loss of alveolar attachments (elastic connective fibres). This causes gas trapping, dynamic hyperinflation, decreased expiratory flow rates and airway collapse, particularly during expiration (Kaufman, 2013). Ms Parker also displayed pursed-lip breathing; this is a technique used to lengthen the expiratory time and improve gaseous exchange, and is a sign of dynamic hyperinflation (Douglas et al, 2013).

In a healthy lung, the destruction and repair of alveolar tissue depends on proteases and antiproteases, mainly released by neutrophils and macrophages. Inhaling cigarette smoke disrupts the usually delicately balanced activity of these enzymes, resulting in the parenchymal damage and small airways (with a lumen of <2mm in diameter) airways disease that is characteristic of emphysema. The severity of parenchymal damage or small airways disease varies, with no pattern related to disease progression (Global Initiative for Chronic Obstructive Lung Disease, 2018).

Ms Parker also had a wheeze, heard through a stethoscope as a continuous whistling sound, which arises from turbulent airflow through constricted airway smooth muscle, a process noted by Mitchell (2015). The wheeze, her 29 pack-year score, exertional breathlessness, cough, sputum production and tiredness, and the findings from her physical examination, were consistent with a diagnosis of COPD (GOLD, 2018; NICE, 2018).

Spirometry is a tool used to identify airflow obstruction but does not identify the cause. Commonly measured parameters are:

• Forced expiratory volume – the volume of air that can be exhaled – in one second (FEV1), starting from a maximal inspiration (in litres);
• Forced vital capacity (FVC) – the total volume of air that can be forcibly exhaled – at timed intervals, starting from a maximal inspiration (in litres).

Calculating the FEV1 as a percentage of the FVC gives the forced expiratory ratio (FEV1/FVC). This provides an index of airflow obstruction; the lower the ratio, the greater the degree of obstruction. In the absence of respiratory disease, FEV1 should be ≥70% of FVC. An FEV1/FVC of <70% is commonly used to denote airflow obstruction (Moore, 2012).

As they are time dependent, FEV1 and FEV1/FVC are reduced in diseases that cause airways to narrow and expiration to slow. FVC, however, is not time dependent: with enough expiratory time, a person can usually exhale to their full FVC. Lung function parameters vary depending on age, height, gender and ethnicity, so the degree of FEV1 and FVC impairment is calculated by comparing a person’s recorded values with predicted values. A recorded value of >80% of the predicted value has been considered ‘normal’ for spirometry parameters but the lower limit of normal – equal to the fifth percentile of a healthy, non-smoking population – based on more robust statistical models is increasingly being used (Cooper et al, 2017).

A reversibility test involves performing spirometry before and after administering a short-acting beta-agonist (SABA) such as salbutamol; the test is used to distinguish between reversible and fixed airflow obstruction. For symptomatic asthma, airflow obstruction due to airway smooth-muscle contraction is reversible: administering a SABA results in smooth-muscle relaxation and improved airflow (Lumb, 2016). However, COPD is associated with fixed airflow obstruction, resulting from neutrophil-driven inflammatory changes, excess mucus secretion and disrupted alveolar attachments, as opposed to airway smooth-muscle contraction.

Administering a SABA for COPD does not usually produce bronchodilation to the extent seen in someone with asthma: a person with asthma may demonstrate significant improvement in FEV1 (of >400ml) after having a SABA, but this may not change in someone with COPD (NICE, 2018). However, a negative response does not rule out therapeutic benefit from long-term SABA use (Marín et al, 2014).

NICE (2018) and GOLD (2018) guidelines advocate performing spirometry after administering a bronchodilator to diagnose COPD. Both suggest a FEV1/FVC of <70% in a person with respiratory symptoms supports a diagnosis of COPD, and both grade the severity of the condition using the predicted FEV1. Ms Parker’s spirometry results showed an FEV1/FVC of 56% and a predicted FEV1 of 57%, with no significant improvement in these values with a reversibility test.

GOLD (2018) guidance is widely accepted and used internationally. However, it was developed by medical practitioners with a medicalised approach, so there is potential for a bias towards pharmacological management of COPD. NICE (2018) guidance may be more useful for practice nurses, as it was developed by a multidisciplinary team using evidence from systematic reviews or meta-analyses of randomised controlled trials, providing a holistic approach. NICE guidance may be outdated on publication, but regular reviews are performed and published online.

NHS England (2016) holds a national register of all health professionals certified in spirometry. It was set up to raise spirometry standards across the country.

Assessment and management

The goals of assessing and managing Ms Parker’s COPD are to:

• Review and determine the level of airflow obstruction;
• Assess the disease’s impact on her life;
• Risk assess future disease progression and exacerbations;
• Recommend pharmacological and therapeutic management.

GOLD’s (2018) ABCD assessment tool (Fig 1) grades COPD severity using spirometry results, number of exacerbations, CAT score and mMRC score, and can be used to support evidence-based pharmacological management of COPD.

When Ms Parker was diagnosed, her predicted FEV1 of 57% categorised her as GOLD grade 2, and her mMRC score, CAT score and exacerbation history placed her in group D. The mMRC scale only measures breathlessness, but the CAT also assesses the impact COPD has on her life, meaning consecutive CAT scores can be compared, providing valuable information for follow-up and management (Zhao, et al, 2014).

After assessing the level of disease burden,  Ms Parker was then provided with education for self-management and lifestyle interventions.

Lifestyle interventions

Smoking cessation.

Cessation of smoking alongside support and pharmacotherapy is the second-most cost-effective intervention for COPD, when compared with most other pharmacological interventions (BTS and PCRS UK, 2012). Smoking cessation:

• Slows the progression of COPD;
• Improves lung function;
• Improves survival rates;
• Reduces the risk of lung cancer;
• Reduces the risk of coronary heart disease risk (Qureshi et al, 2014).

Ms Parker accepted a referral to an All Wales Smoking Cessation Service adviser based at her GP surgery. The adviser used the internationally accepted ‘five As’ approach:

• Ask – record the number of cigarettes the individual smokes per day or week, and the year they started smoking;
• Advise – urge them to quit. Advice should be clear and personalised;
• Assess – determine their willingness and confidence to attempt to quit. Note the state of change;
• Assist – help them to quit. Provide behavioural support and recommend or prescribe pharmacological aids. If they are not ready to quit, promote motivation for a future attempt;
• Arrange – book a follow-up appointment within one week or, if appropriate, refer them to a specialist cessation service for intensive support. Document the intervention.

NICE (2013) guidance recommends that this be used at every opportunity. Stead et al (2016) suggested that a combination of counselling and pharmacotherapy have proven to be the most effective strategy.

Pulmonary rehabilitation

Ms Parker’s positive response to smoking cessation provided an ideal opportunity to offer her pulmonary rehabilitation (PR)  – as indicated by Johnson et al (2014), changing one behaviour significantly increases a person’s chance of changing another.

PR – a supervised programme including exercise training, health education and breathing techniques – is an evidence-based, comprehensive, multidisciplinary intervention that:

• Improves exercise tolerance;
• Reduces dyspnoea;
• Promotes weight loss (Bolton et al, 2013).

These improvements often lead to an improved quality of life (Sciriha et al, 2015).

Most relevant for Ms Parker, PR has been shown to reduce anxiety and depression, which are linked to an increased risk of exacerbations and poorer health status (Miller and Davenport, 2015). People most at risk of future exacerbations are those who already experience them (Agusti et al, 2010), as in Ms Parker’s case. Patients who have frequent exacerbations have a lower quality of life, quicker progression of disease, reduced mobility and more-rapid decline in lung function than those who do not (Donaldson et al, 2002).

“COPD is a major public-health challenge; nurses can be pivotal in early identification, assessment and management”

Pharmacological interventions

Ms Parker has been prescribed inhaled salbutamol as required; this is a SABA that mediates the increase of cyclic adenosine monophosphate in airway smooth-muscle cells, leading to muscle relaxation and bronchodilation. SABAs facilitate lung emptying by dilatating the small airways, reversing dynamic hyperinflation of the lungs (Thomas et al, 2013). Ms Parker also uses a long-acting muscarinic antagonist (LAMA) inhaler, which works by blocking the bronchoconstrictor effects of acetylcholine on M3 muscarinic receptors in airway smooth muscle; release of acetylcholine by the parasympathetic nerves in the airways results in increased airway tone with reduced diameter.

At a routine review, Ms Parker admitted to only using the SABA and LAMA inhalers, despite also being prescribed a combined inhaled corticosteroid and long-acting beta 2 -agonist (ICS/LABA) inhaler. She was unaware that ICS/LABA inhalers are preferred over SABA inhalers, as they:

• Last for 12 hours;
• Improve the symptoms of breathlessness;
• Increase exercise tolerance;
• Can reduce the frequency of exacerbations (Agusti et al, 2010).

However, moderate-quality evidence shows that ICS/LABA combinations, particularly fluticasone, cause an increased risk of pneumonia (Suissa et al, 2013; Nannini et al, 2007). Inhaler choice should, therefore, be individualised, based on symptoms, delivery technique, patient education and compliance.

It is essential to teach and assess inhaler technique at every review (NICE, 2011). Ms Parker uses both a metered-dose inhaler and a dry-powder inhaler; an in-check device is used to assess her inspiratory effort, as different inhaler types require different inhalation speeds. Braido et al (2016) estimated that 50% of patients have poor inhaler technique, which may be due to health professionals lacking the confidence and capability to teach and assess their use.

Patients may also not have the dexterity, capacity to learn or vision required to use the inhaler. Online resources are available from, for example, RightBreathe (rightbreathe.com), British Lung Foundation (blf.org.uk). Ms Parker’s adherence could be improved through once-daily inhalers, as indicated by results from a study by Lipson et al (2017). Any change in her inhaler would be monitored as per local policy.

Vaccinations

Ms Parker keeps up to date with her seasonal influenza and pneumococcus vaccinations. This is in line with the low-cost, highest-benefit strategy identified by the British Thoracic Society and Primary Care Respiratory Society UK’s (2012) study, which was conducted to inform interventions for patients with COPD and their relative quality-adjusted life years. Influenza vaccinations have been shown to decrease the risk of lower respiratory tract infections and concurrent COPD exacerbations (Walters et al, 2017; Department of Health, 2011; Poole et al, 2006).

Self-management

Ms Parker was given a self-management plan that included:

• Information on how to monitor her symptoms;
• A rescue pack of antibiotics, steroids and salbutamol;
• A traffic-light system demonstrating when, and how, to commence treatment or seek medical help.

Self-management plans and rescue packs have been shown to reduce symptoms of an exacerbation (Baxter et al, 2016), allowing patients to be cared for in the community rather than in a hospital setting and increasing patient satisfaction (Fletcher and Dahl, 2013).

Improving Ms Parker’s adherence to once-daily inhalers and supporting her to self-manage and make the necessary lifestyle changes, should improve her symptoms and result in fewer exacerbations.

The earlier a diagnosis of COPD is made, the greater the chances of reducing lung damage through interventions such as smoking cessation, lifestyle modifications and treatment, if required (Price et al, 2011).

• Chronic obstructive pulmonary disease is a progressive respiratory condition, projected to become the third leading cause of death globally
• Diagnosis involves taking a patient history and performing spirometry testing
• Spirometry identifies airflow obstruction by measuring the volume of air that can be exhaled
• Chronic obstructive pulmonary disease is managed with lifestyle and pharmacological interventions, as well as self-management

Related files

200506 diagnosis and management of copd – a case study.

• Add to Bookmarks

Related articles

Nurse-led cognitive behavioural therapy for respiratory patients

Anxiety and depression are common comorbidities of chronic obstructive pulmonary disease. This…

Issues of home-based non-invasive ventilation

Non-invasive ventilation is increasingly used to manage patients with COPD at home,…

Improving outcomes with online COPD self-care

An innovative approach to the self-management of chronic obstructive pulmonary disease is…

An audit of care provided to patients with COPD

Nationwide audit of COPD care reveals many aspects of provision have improved,…

Have your say

Sign in or Register a new account to join the discussion.

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

• View all journals
• Explore content
• About the journal
• Publish with us
• Sign up for alerts
• Perspective
• Open access
• Published: 07 May 2015

Four patients with a history of acute exacerbations of COPD: implementing the CHEST/Canadian Thoracic Society guidelines for preventing exacerbations

• Ioanna Tsiligianni 1 , 2 ,
• Donna Goodridge 3 ,
• Darcy Marciniuk 4 ,
• Sally Hull 5 &
• Jean Bourbeau 6  

npj Primary Care Respiratory Medicine volume  25 , Article number:  15023 ( 2015 ) Cite this article

60k Accesses

12 Altmetric

Metrics details

• Respiratory tract diseases

The American College of Chest Physicians and Canadian Thoracic Society have jointly produced evidence-based guidelines for the prevention of exacerbations in chronic obstructive pulmonary disease (COPD). This educational article gives four perspectives on how these guidelines apply to the practical management of people with COPD. A current smoker with frequent exacerbations will benefit from support to quit, and from optimisation of his inhaled treatment. For a man with very severe COPD and multiple co-morbidities living in a remote community, tele-health care may enable provision of multidisciplinary care. A woman who is admitted for the third time in a year needs a structured assessment of her care with a view to stepping up pharmacological and non-pharmacological treatment as required. The overlap between asthma and COPD challenges both diagnostic and management strategies for a lady smoker with a history of asthma since childhood. Common threads in all these cases are the importance of advising on smoking cessation, offering (and encouraging people to attend) pulmonary rehabilitation, and the importance of self-management, including an action plan supported by multidisciplinary teams.

Similar content being viewed by others

Diagnostic spirometry in COPD is increasing, a comparison of two Swedish cohorts

Phenotype and management of chronic obstructive pulmonary disease patients in general population in China: a nationally cross-sectional study

Cardiovascular predictors of mortality and exacerbations in patients with COPD

Case study 1: a 63-year-old man with moderate/severe copd and a chest infection.

A 63-year-old self-employed plumber makes a same-day appointment for another ‘chest infection’. He caught an upper respiratory tract infection from his grandchildren 10 days ago, and he now has a productive cough with green sputum, and his breathlessness and fatigue has forced him to take time off work.

He has visited his general practitioner with similar symptoms two or three times every year in the last decade. A diagnosis of COPD was confirmed 6 years ago, and he was started on a short-acting β 2 -agonist. This helped with his day-to-day symptoms, although recently the symptoms of breathlessness have been interfering with his work and he has to pace himself to get through the day. Recovering from exacerbations takes longer than it used to—it is often 2 weeks before he is able to get back to work—and he feels bad about letting down customers. He cannot afford to retire, but is thinking about reducing his workload.

He last attended a COPD review 6 months ago when his FEV 1 was 52% predicted. He was advised to stop smoking and given a prescription for varenicline, but he relapsed after a few days and did not return for the follow-up appointment. He attends each year for his ‘flu vaccination’. His only other medication is an ACE inhibitor for hypertension.

Managing the presenting problem. Is it a COPD exacerbation?

A COPD exacerbation is defined as ‘an acute event characterised by a worsening of the patient’s respiratory symptoms that is beyond normal day-to-day variation and leads to change in medications’. 1 , 2 The worsening symptoms are usually increased dyspnoea, increased sputum volume and increased sputum purulence. 1 , 2 All these symptoms are present in our patient who experiences an exacerbation triggered by a viral upper respiratory tract infection—the most common cause of COPD exacerbations. Apart from the management of the acute exacerbation that could include antibiotics, oral steroids and increased use of short-acting bronchodilators, special attention should be given to his on-going treatment to prevent future exacerbations. 2 Short-term use of systemic corticosteroids and a course of antibiotics can shorten recovery time, improve lung function (forced expiratory volume in one second (FEV 1 )) and arterial hypoxaemia and reduce the risk of early relapse, treatment failure and length of hospital stay. 1 , 2 Short-acting inhaled β 2 -agonists with or without short-acting anti-muscarinics are usually the preferred bronchodilators for the treatment of an acute exacerbation. 1

Reviewing his routine treatment

One of the concerns about this patient is that his COPD is inadequately treated. The Global Initiative for Chronic Obstructive Lung Disease (GOLD) suggests that COPD management be based on a combined assessment of symptoms, GOLD classification of airflow limitation, and exacerbation rate. 1 The modified Medical Research Council (mMRC) dyspnoea score 3 or the COPD Assessment Tool (CAT) 4 could be used to evaluate the symptoms/health status. History suggests that his breathlessness has begun to interfere with his lifestyle, but this has not been formally asssessed since the diagnosis 6 years ago. Therefore, one would like to be certain that these elements are taken into consideration in future management by involving other members of the health care team. The fact that he had two to three exacerbations per year puts the patient into GOLD category C–D (see Figure 1 ) despite the moderate airflow limitation. 1 , 5 Our patient is only being treated with short-acting bronchodilators; however, this is only appropriate for patients who belong to category A. Treatment options for patients in category C or D should include long-acting muscarinic antagonists (LAMAs) or long-acting β 2 -agonists (LABAs), which will not only improve his symptoms but also help prevent future exacerbations. 2 Used in combination with LABA or LAMA, inhaled corticosteroids also contribute to preventing exacerbations. 2

The four categories of COPD based on assessment of symptoms and future risk of exacerbations (adapted by Gruffydd-Jones, 5 from the Global Strategy for Diagnosis, Management and Prevention of COPD). 1 CAT, COPD Assessment Tool; COPD, chronic obstructive pulmonary disease; mMRC, modified Medical Research Council Dyspnoea Scale.

Prevention of future exacerbations

Exacerbations should be prevented as they have a negative impact on the quality of life; they adversely affect symptoms and lung function, increase economic cost, increase mortality and accelerate lung function decline. 1 , 2 Figure 2 summarises the recommendations and suggestions of the joint American College of Chest Physicians and Canadian Thoracic Society (CHEST/CTS) Guidelines for the prevention of exacerbations in COPD. 2 The grades of recommendation from the CHEST/CTS guidelines are explained in Table 1 .

Decision tree for prevention of acute exacerbations of COPD (reproduced with permission from the CHEST/CTS Guidelines for the prevention of exacerbations in COPD). 2 This decision tree for prevention of acute exacerbations of COPD is arranged according to three key clinical questions using the PICO format: non-pharmacologic therapies, inhaled therapies and oral therapies. The wording used is ‘Recommended or Not recommended’ when the evidence was strong (Level 1) or ‘Suggested or Not suggested’ when the evidence was weak (Level 2). CHEST/CTS, American College of Chest Physicians and Canadian Thoracic Society; COPD, chronic obstructive pulmonary disease; FEV 1 , forced expiratory volume in one second; FVC, forced vital capacity; LABA, long-acting β-agonist; LAMA, long-acting muscarinic antagonist; ICS, inhaled corticosteroids; SAMA, short-acting muscarinic antagonist; SABA, short-acting β-agonist; SM, self-management.

Pharmacological approach

In patients with moderate-to-severe COPD, the use of LABA or LAMA compared with placebo or short-acting bronchodilators is recommended to prevent acute exacerbations (Grades 1B and 1A, respectively). 2 , 6 , 7 LAMAs are associated with a lower rate of exacerbations compared with LABAs (Grade 1C). 2 , 6 The inhaler technique needs to be checked and a suitable device selected. If our patient does not respond to optimizing inhaled medication and continues to have two to three exacerbations per year, there are additional options that offer pulmonary rehabilitation and other forms of pharmacological therapy, such as a macrolide, theophylline, phosphodieseterase (PDE4) inhibitor or N -acetylocysteine/carbocysteine, 2 although there is no information about their relative effectiveness and the order in which they should be prescribed. The choice of prescription should be guided by the risk/benefit for a given individual, and drug availability and/or cost within the health care system.

Non-pharmacological approach

A comprehensive patient-centred approach based on the chronic care model could be of great value. 2 , 8

This should include the following elements

Vaccinations: the 23-valent pneumococcal vaccine and annual influenza vaccine are suggested as part of the overall medical management in patients with COPD. 2 Although there is no clear COPD-specific evidence for the pneumococcal vaccine and the evidence is modest for influenza, the CHEST/CTS Guidelines concur with advice of the World Health Organization (WHO) 9 and national advisory bodies, 10 – 12 and supports their use in COPD patients who are at risk for serious infections. 2

Smoking cessation (including counselling and treatment) has low evidence for preventing exacerbations (Grade 2C). 2 However, the benefits from smoking cessation are outstanding as it improves COPD prognosis, slows lung function decline and improves the quality of life and symptoms. 1 , 2 , 13 , 14 Our patient has struggled to quit in the past; assessing current readiness to quit, and encouraging and supporting a future attempt is a priority in his care.

Pulmonary rehabilitation (based on exercise training, education and behaviour change) in people with moderate-to-very-severe COPD, provided within 4 weeks of an exacerbation, can prevent acute exacerbations (Grade 1C). 2 Pulmonary rehabilitation is also an effective strategy to improve symptoms, the quality of life and exercise tolerance, 15 , 16 and our patient should be encouraged to attend a course.

Self-management education with a written action plan and supported by case management providing regular direct access to a health care specialist reduces hospitalisations and prevents severe acute exacerbations (Grade 2C). 2 Some patients with good professional support can have an emergency course of steroids and antibiotics to start at the onset of an exacerbation in accordance with their plan.

Finally, close follow-up is needed for our patient as he was inadequately treated, relapsed from smoking cessation after a few days despite varenicline, and missed his follow-up appointment. A more alert health care team may have been able to identify these issues, avoid his relapse and take a timely approach to introducing additional measures to prevent his recurrent acute exacerbations.

Case study 2: A 74-year-old man with very severe COPD living alone in a remote community

A 74-year-old man has a routine telephone consultation with the respiratory team. He has very severe COPD (his FEV 1 2 years ago was 24% of predicted) and he copes with the help of his daughter who lives in the same remote community. He quit smoking the previous year after an admission to the hospital 50 miles away, which he found very stressful. He and his family managed another four exacerbations at home with courses of steroids and antibiotics, which he commenced in accordance with a self-management plan provided by the respiratory team.

His usual therapy consists of regular long-acting β 2 -agonist/inhaled steroid combination and a long-acting anti-muscarinic. He has a number of other health problems, including coronary heart disease and osteoarthritis and, in recent times, his daughter has become concerned that he is becoming forgetful. He manages at home by himself, steadfastly refusing social help and adamant that he does not want to move from the home he has lived in for 55 years.

This is a common clinical scenario, and a number of important issues require attention, with a view to optimising the management of this 74-year-old man suffering from COPD. He has very severe obstruction, is experiencing frequent acute flare-ups, is dependent and isolated and has a number of co-morbidities. To work towards preventing future exacerbations in this patient, a comprehensive plan addressing key medical and self-care issues needs to be developed that accounts for his particular context.

Optimising medical management

According to the CHEST/CTS Guidelines for prevention of acute exacerbations of COPD, 2 this patient should receive an annual influenza vaccination and may benefit from a 23-valent pneumococcal vaccine (Grades 1B and 2C, respectively). Influenza infection is associated with greater risk of mortality in COPD, as well as increased risk of hospitalisation and disease progression. 1 A diagnosis of COPD also increases the risk for pneumococcal disease and related complications, with hospitalisation rates for patients with COPD being higher than that in the general population. 10 , 17 Although existing evidence does not support the use of this vaccine specifically to prevent exacerbations of COPD, 1 administration of the 23-valent pneumococcal vaccine is recommended as a component of overall medical management. 9 – 12

Long-term oxygen therapy has been demonstrated to improve survival in people with chronic hypoxaemia; 18 it would be helpful to obtain oxygen saturation levels and consider whether long-term oxygen therapy would be of benefit to this patient.

Even though this patient is on effective medications, further optimisation of pharmacologic therapy should be undertaken, including reviewing administration technique for the different inhaler devices. 19 Maintenance PDE4 inhibitors, such as roflumilast or theophyllines, long-term macrolides (i.e., azithromycin) or oral N -acetylcysteine are potential considerations. Each of these therapeutic options has demonstrated efficacy in preventing future acute exacerbations, although they should be used with caution in this frail elderly man. 2 This patient would benefit from a review of co-morbidities, including a chest X-ray, electrocardiogram, memory assessment and blood tests including haemoglobin, glucose, thyroid and renal function assessments.

Pulmonary rehabilitation, supported self-management and tele-health care

Pulmonary rehabilitation for patients who have recently experienced an exacerbation of COPD (initiated <4 weeks following the exacerbation) has been demonstrated to prevent subsequent exacerbations (Grade 1C). 2 Existing evidence suggests that pulmonary rehabilitation does not reduce future exacerbations when the index exacerbation has occurred more than 4 weeks earlier; 2 however, its usefulness is evident in other important patient-centred outcomes such as improved activity, walking distance and quality of life, as well as by reduced shortness of breath. It would be appropriate to discuss this and enable our patient to enrol in pulmonary rehabilitation.

The patient’s access to pulmonary rehabilitation in his remote location, however, is likely to be limited. Several reports have noted that only one to two percent of people with COPD are able to access pulmonary rehabilitation programmes within Canada, 20 the United States 21 and the United Kingdom. 22 Alternatives to hospital-based pulmonary rehabilitation programmes, such as home-based programmes or programmes offered via tele-health, may be options for this patient. 23 Home-based pulmonary rehabilitation programmes have been found to improve exercise tolerance, symptom burden and quality of life. 24 – 27 Outcomes of a pulmonary rehabilitation programme offered via tele-health have also been found to be comparable to those of a hospital-based programme, 28 and may be worth exploring.

Written self-management (action) plans, together with education and case management, are suggested in the CHEST/CTS guidelines as a strategy to reduce hospitalisation and emergency department visits attributable to exacerbations of COPD (Grade 2B). 2 Our patient has an existing action plan, which has enabled him and his family to manage some exacerbations at home. Although the patient has likely had some education on COPD and its management in the past, on-going reinforcement of key principles may be helpful in preventing future exacerbations.

The self-management plan should be reviewed regularly to ensure the advice remains current. The patient’s ability to use the self-management plan safely also needs to be assessed, given his daughter’s recent observation of forgetfulness and his living alone. Cognitive impairment is being increasingly recognised as a significant co-morbidity of COPD. 29 , 30 Patients who were awaiting discharge from hospital following an exacerbation of COPD were found to perform significantly worse on a range of cognitive functional measures than a matched group with stable COPD, a finding that persisted 3 months later. 29 Cognitive impairment may contribute independently to the risk for future exacerbations by increasing the likelihood of incorrect inhaler device use and failure to adhere to recommended treatments. 29

Given that this patient resides in a remote location, access to case management services that assist in preventing future exacerbations may be difficult or impossible to arrange. Although there is currently insufficient evidence that in general the use of telemonitoring contributes to the prevention of exacerbations of COPD, 2 tele-health care for this remotely located patient has potential to allow for case management at a distance, with minimal risk to the patient. Further study is needed to address this potential benefit.

Assessing for and managing frailty

Recognising this patient’s co-morbid diagnoses of coronary heart disease and osteoarthritis, careful assessment of functional and self-care abilities would be appropriate. Almost 60% of older adults with COPD meet the criteria for frailty. 31 Frailty is defined as a dynamic state associated with decline of physiologic reserves in multiple systems and inability to respond to stressful insults. 32 Frailty is associated with an increased risk for institutionalisation and mortality. 33 , 34 Given the complex needs of those who are frail, screening this patient for frailty would constitute patient-centred and cost-effective care. Frailty assessment tools, such as the seven-point Clinical Frailty Index, 35 may provide structure to this assessment.

Admission to a hospital 50 miles away from our patient’s home last year for an exacerbation was stressful. Since his hospitalisation, this patient has experienced four additional exacerbations that have been managed at home in his remote community. It would be appropriate to explore the patient’s treatment wishes and determine whether the patient has chosen to refuse further hospitalisations. Our patient’s risk of dying is significant, with risk factors increasing the risk of short-term mortality following an exacerbation of COPD (GOLD Stage 4, age, male sex, confusion). 36 Mortality rates between 22 and 36% have been documented in the first and second years, respectively, following an exacerbation, 37 , 38 which also increase with the frequency and severity of hospitalisations. 39

Our patient has refused social help and does not want to be relocated from his home. Ageing in their own home is a key goal of many older adults. 40 , 41 Efforts to ensure that adequate resources to support the patient are available (and to support the daughter who is currently providing a lot of his care) will form an important part of the plan of care.

Case study 3: A 62-year-old woman with severe COPD admitted with an exacerbation

A 62-year-old lady is admitted for the third time this year with an acute exacerbation of her severe COPD. Her FEV 1 was 35% predicted at the recent outpatient visit. She retired from her job as a shop assistant 5 years ago because of her breathlessness and now devotes her time to her grandchildren who ‘exhaust her’ but give her a lot of pleasure.

She quit smoking 5 years ago. Over the years, her medication has increased, as nothing seemed to relieve her uncomfortable breathlessness, and, in addition to inhaled long-acting β 2 -agonist/ inhaled steroid combination and a long-acting anti-muscarinic, she is taking theophylline and carbocysteine, although she is not convinced of their beneficial effect. Oral steroid courses help her dyspnoea and she has taken at least six courses this year: she has an action plan and keeps an emergency supply of medication at home.

A secondary care perspective on the management strategy for this woman

Acute exacerbations of COPD have serious negative consequences for health care systems and patients. The risk of future events and complications, such as hospital admission and poor patient outcomes (disability and reduced health status), can be improved through a combination of non-pharmacological and pharmacological therapies. 2

Evaluation of the patient, risk assessment and adherence to medication

The essential first step in the management of this lady (as for any patient) includes a detailed medical evaluation. Our patient has a well-established diagnosis of COPD with severe airflow obstruction (GOLD grade 3), significant breathlessness that resulted in her retiring from her job, and recurrent exacerbations. She does not have significant co-morbidity, although this requires to be confirmed. Further to the medical evaluation, it is important to assess her actual disease management (medication and proper use) as well as making sure she has adopted a healthy lifestyle (smoking cessation, physical activities and exercise). Does she live in a smoke-free environment? Effect of and evidence for smoking cessation in the prevention of acute exacerbations of COPD is low, but evidence exists for a reduction in cough and phlegm after smoking cessation and less lung function decline upon sustained cessation. With respect to the medication, never assume that it is taken as prescribed. When asking the patient, use open questions such as ‘I would like to hear how you take your medication on a typical day?’ instead of ‘Did you take the medication as prescribed’. Open questions tend to elicit more useful and pertinent information, and invite collaboration. Asking the patient to demonstrate her inhalation technique shows you the way she uses her different inhalation devices.

Optimising the pharmacological therapy

The second step is to assess whether the patient is on optimal treatment to prevent exacerbations. In other words, can we do better helping the patient manage her disease and improving her well-being. As in the previous cases, vaccination, in particular, annual administration of the influenza vaccine, should be prescribed for this lady. We should evaluate other alternatives of pharmacological therapy that could improve symptoms, prevent exacerbations and reduce the use of repeated systemic corticosteroids with their important adverse effects (such as osteoporosis, cataracts, diabetes). Prescribing a PDE4 inhibitor (Grade 2A) or a long-term macrolide (Grade 2A) once a day would be a consideration for this lady. 2 As there is no superiority trial comparing these two medications, our preference will be based on potential side effects, as well as cost and access to treatment. For PDE4 inhibitors, there are limited data on supplemental effectiveness in patients with COPD and chronic bronchitis concurrently using inhaled therapies, and they potentially have side effects such as diarrhoea, nausea, headache and weight loss. The side effects tend to diminish over time, but some patients may have to discontinue the therapy. Long-term macrolides have been studied in COPD patients already treated with inhaled therapies and shown to be effective, although clinicians need to consider in their individual patients the potential for harm, such as prolongation of the QT interval, hearing loss and bacterial resistance. Furthermore, the duration (beyond 1 year) and exact dosage of macrolide therapy (for example, once daily versus three times per week) are unknown.

Making non-pharmacological therapy an essential part of the management

The third step, often neglected in the management of COPD patients, is non-pharmacological therapy. For this lady, we suggest self-management education with a written action plan and case management to improve how she deals with exacerbations (Grade 2B). 2 The expectation will not be to reduce exacerbations but to prevent emergency department visits and hospital admissions. However, despite general evidence of efficacy, 42 not all self-management interventions have been shown to be effective or to benefit all COPD patients 43 , 44 (some have been shown to be potentially harmful 44 ). The effectiveness of any complex intervention such as self-management in COPD critically depends on the health care professionals who deliver the intervention, as well on the patient and the health care system. The patient may not have the motivation or desire to change or to commit to an intensive programme. The individual patient’s needs, preferences and personal goals should inform the design of any intervention with a behavioural component. For this lady, it is essential to apply integrated disease management and to refer the patient to a pulmonary rehabilitation programme. Pulmonary rehabilitation has high value, including reducing the risk for hospitalisation in COPD patients with recent exacerbations (Grade 1C). 2 The most important benefits our patient can expect from participating in structured supervised exercise within pulmonary rehabilitation are improved health status, exercise tolerance and a reduction in dyspnoea (Grade 1A). 2 , 15 Pulmonary rehabilitation programmes provide clinicians with an opportunity to deliver education and self-management skills to patients with COPD, and are well established as a means of enhancing standard therapy to control and alleviate symptoms, optimise functional capacity and improve health-related quality of life.

Case study 4: A 52-year-old lady with moderate COPD—and possibly asthma

A 52-year-old lady attends to discuss her COPD and specifically the problem she is having with exacerbations and time ‘off sick’. She is a heavy smoker, and her progressively deteriorating lung function suggests that she has moderate COPD, although she also has a history of childhood asthma, and had allergic rhinitis as a teenager. Recent spirometry showed a typical COPD flow-volume loop, although she had some reversibility (250 ml and 20%) with a post-bronchodilator FEV 1 of 60% predicted.

She has a sedentary office job and, although she is breathless on exertion, this generally does not interfere with her lifestyle. The relatively frequent exacerbations are more troublesome. They are usually triggered by an upper respiratory infection and can take a couple of weeks to recover. She has had three exacerbations this winter, and as a result her employer is not happy with her sickness absence record and has asked her to seek advice from her general practitioner.

She has a short-acting β 2 -agonist, although she rarely uses it except during exacerbations. In the past, she has used an inhaled steroid, but stopped that some time ago as she was not convinced it was helping.

It is a welcome opportunity when a patient comes to discuss her COPD with a particular issue to address. With a history of childhood asthma, and serial COPD lung function tests, she has probably been offered many components of good primary care for COPD, but has not yet fully engaged with her management. We know that ~40% of people with COPD continue to smoke, and many are intermittent users of inhaled medications. 45 It is easy to ignore breathlessness when both job and lifestyle are sedentary.

Understanding her diagnosis and setting goals

Her readiness to engage can be supported by a move to structured collaborative care, enabling the patient to have the knowledge, resources and support to make the necessary changes. Much of this can be done by the primary care COPD team, including the pharmacist. Regular recall to maintain engagement is essential.

The combination of childhood asthma, rhinitis and a long history of smoking requires diagnostic review. This might include serial peak flows over 2 weeks to look for variability, and a chest X-ray, if not done recently, to rule out lung cancer as a reason for recent exacerbations. Her spirometry suggests moderate COPD, 1 , 46 but she also has some reversibility, not enough to place her in the asthma camp but, combined with her past medical history, being enough to explore an asthma COPD overlap syndrome. This is important to consider as it may guide decisions on inhaled medication, and there is evidence that lung function deteriorates faster in this group. 47 It is estimated that up to 20% of patients have overlap diagnoses, although the exact prevalence depends on the definition. 48

Reducing the frequency of exacerbations

Exacerbations in COPD are debilitating, often trigger hospital admission and hasten a progressive decline in pulmonary function. 2 Written information on interventions that can slow down the course of COPD and reduce the frequency and impact of exacerbations will help to support progressive changes in management.

Smoking cessation

Few people are unaware that cessation of smoking is the key intervention for COPD. Reducing further decline in lung function will slow down the severity of exacerbations. Finding a smoking cessation programme that suits her working life, exploring previous attempts at cessation, offering pharmacotherapy and a non-judgemental approach to further attempts at stopping are crucial.

Immunisations

Many, but not all, exacerbations of COPD are triggered by viral upper respiratory tract infections. Annual flu immunisation is a part of regular COPD care and reduces exacerbations and hospitalisation when flu is circulating (Grade 1B). Pneumococcal immunisation should be offered, although evidence for reducing exacerbations is weak; those with COPD will be at greater risk for pneumococcal infection. 2

Pulmonary rehabilitation

Pulmonary rehabilitation improves symptoms, quality of life and reduces hospital admission. 49 It is most efficacious in patients who are symptomatic (MRC dyspnoea scale 3 and above) and in terms of reducing exacerbations is most effective when delivered early after an exacerbation (Grade 1C). 2 The major hurdle is encouraging patients to attend, with most programmes showing an attrition rate of 30% before the first appointment, and high rates of non-completion. 45 , 50 Effective programmes that maintain the gains from aerobic exercise are more cost-effective than some of the inhaled medications in use (see Figure 3 ). 50

The COPD value pyramid (developed by the London Respiratory Network with The London School of Economics and reproduced with permission from the London Respiratory Team report 2013). 48 This 'value' pyramid reflects what we currently know about the cost per QALY of some of the commonest interventions in COPD. It was devised as a tool for health care organisations to use to promote audit and to ensure adequate commissioning of non-pharmacological interventions. COPD, chronic obstructive pulmonary disease; LABA, long-acting β-agonist; QALY, quality-adjusted life-year.

Inhaled medication is likely to improve our patient’s breathlessness and contribute to a reduction in exacerbation frequency. Currently, she uses only a short-acting β 2 -agonist. One wonders if she has a spacer? How much of the medicine is reaching her lungs? Repeated observation and training in inhaler use is essential if patients are to benefit from expensive medications.

With her history of asthma and evidence of some reversibility, the best choice of regular medication may be a combination of inhaled corticosteroid and a LABA. Guidelines suggest the asthma component in asthma COPD overlap syndrome should be the initial treatment target, 48 and a LABA alone should be avoided. Warn about oral thrush, and the increased risk for pneumonia. 46 If she chooses not to use an inhaled steroid, then a trial of a LAMA is indicated. Both drugs reduce exacerbation rates. 2 , 51

Finally, ensuring early treatment of exacerbations speeds up recovery. 52 Prescribe rescue medication (a 5–7-day course of oral steroids and antibiotic) to be started when symptomatic, and encourage attendance at a post-exacerbation review.

Global Initiative for Chronic Obstructive Lung Disease (GOLD). Global Strategy for the Diagnosis, Management and Prevention of COPD. Updated 2014. http://www.goldcopd.org. Accessed January 2015.

Criner GJ, Bourbeau J, Diekemper RL, Ouellette DR, Goodridge D, Hernandez P et al. Prevention of acute exacerbations of chronic obstructive pulmonary disease: American College of Chest Physicians and Canadian Thoracic Society Guideline. Chest 2015; 147 : 894–942.

Article   Google Scholar  

Fletcher CM . Standardised questionnaire on respiratory symptoms: a statement prepared and approved by the MRC Committee on the Aetiology of Chronic Bronchitis (MRC breathlessness score). BMJ 1960; 2 : 1665.

Google Scholar  

Jones PW, Harding G, Berry P, Wiklund I, Chen W-H, Leidy NK . Development and first validation of the COPD Assessment Test. Eur Respir J 2009; 34 : 648–654.

Article   CAS   Google Scholar  

Gruffydd-Jones K . GOLD guidelines 2011: what are the implications for primary care? Prim Care Respir J 2012; 21 : 437–441.

Chong J, Karner C, Poole P . Tiotropium versus long-acting beta-agonists for stable chronic obstructive pulmonary disease. Cochrane Database Syst Rev 2012; 9 : CD009157.

Cheyne L, Irvin-Sellers MJ, White J . Tiotropium versus ipratropium bromide for chronic obstructive pulmonary disease. Cochrane Database Syst Rev 2013; 9 : CD009552.

Bodenheimer T, Wagner EH, Grumbach K . Improving primary care for patients with chronic illness. JAMA 2002; 288 : 1775–1779.

World Health Organization. 23-valent pneumococcal polysaccharide vaccine. WHO position paper. Wkly Epidemiol Rec 2008; 83 : 373–384.

Center for Disease Control and Prevention Pneumococcal vaccination. http://www.cdc.gov/vaccines/vpd-vac/pneumo/ . Accessed January 2015.

Public Health England (2014a) Immunisation against infectious disease - "The Green Book". Chapter 19 - Influenza. https://www.gov.uk/government/collections/immunisation-against-infectious-disease-the-green-book . Accessed January 2015.

Public Health Agency of Canada. Canadian immunization guide: Pneumococcal vaccine, 2014. http://www.phac-aspc.gc.ca/publicat/cig-gci/p04-pneu-eng.php . Accessed January 2015.

Fletcher C, Peto R . The natural history of chronic airflow obstruction. Br Med J 1977; 1 : 1645–1648.

Hersh CP, DeMeo DL, Al-Ansari E, Carey VJ, Reilly JJ, Ginns LC et al. Predictors of survival in severe, early onset COPD. Chest 2004; 126 : 1443–1451.

Lacasse Y, Goldstein R, Lasserson TJ, Martin S . Pulmonary rehabilitation for chronic obstructive pulmonary disease. Cochrane Database Syst Rev 2006; (4): CD003793.

Lacasse Y, Wong E, Guyatt GH, King D, Cook DJ, Goldstein RS . Meta-analysis of respiratory rehabilitation in chronic obstructive pulmonary disease. Lancet 1996; 348 : 1115–1119.

Lee T, Weaver F, Weiss K . Impact of pneumococcal vaccination on pneumonia rates in patients with COPD and asthma. J Gen Intern Med 2007; 22 : 62–67.

Crockett AJ, Cranston JM, Moss JR, Allpers JH . A review of long-term oxygen therapy for chronic obstructive pulmonary disease. Respir Med 2001; 95 : 437–443.

Newman SP . Inhaler treatment options in COPD. Eur Respir Rev 2005; 96 : 102–108.

Brooks D, Sottana R, Bell B, Hanna M, Laframboise L, Selvanayagarajah S et al. Characterization of pulmonary rehabilitation programs in Canada in 2005. Can Respir J 2007; 14 : 87–92.

Bickford LS, Hodgkin JE, McInturff SL . National pulmonary rehabilitation survey. Update. J Cardiopulm Rehabil 1995; 15 : 406–411.

Yohannes AM, Connolly MJ . Pulmonary rehabilitation programmes in the UK: a national representative survey. Clin Rehabil 2004; 18 : 444–449.

Marciniuk DD, Brooks D, Butcher S, Debigare R, Dechman G, Ford G et al. Optimizing pulmonary rehabilitation in chronic obstructive pulmonary disease – practical issues: a Canadian Thoracic Society Clinical Practice Guideline. Can Respir J 2010; 17 : 159–168.

Guell M, De-Lucas P, Galdiz J, Montemayor T, Rodriguez Gonzalez-Moro J, Gorostiza A . Home vs hospital-based pulmonary rehabilitation for patients with chronic obstructive pulmonary disease: disease: a Spanish multicentre trial. Arch Bronconeumol 2008; 44 : 512–518.

Maltais F, Bourbeau J, Shapiro S, Lacasse Y, Perrault H, Baltzan M . Effects of home-based pulmonary rehabilitation in patients with chronic obstructive pulmonary disease: a randomized trial. Ann Intern Med 2008; 149 : 869–878.

Puente-Maestu L, Sanz M, Sanz P, Cubillo J, Mayol J, Casaburi R . Comparison of effects of supervised versus self-monitored training programmes in patients with chronic obstructive pulmonary disease. Eur Respir J 2000; 15 : 517–525.

Strijbos J, Postma D, Van-Altena R, Gimeno F, Koeter G . A comparison between an outpatient hospital-based pulmonary rehabilitation program and a home-care pulmonary rehabilitation program in patients with COPD. A follow-up of 18 months. Chest 1996; 109 : 366–372.

Stickland M, Jourdain T, Wong EY, Rodgers WM, Jendzjowsky NG, MacDonald GF . Using Telehealth technology to deliver pulmonary rehabilitation in chronic obstructive pulmonary disease patients. Can Respir J 2011; 18 : 216–220.

Dodd JW, Getov SV, Jones PW . Cognitive function in COPD. Eur Respir J 2010; 35 : 913–922.

Cleutjens FA, Spruit MA, Ponds RW, Dijkstra JB, Franssen FM, Wouters EF et al. Cognitive functioning in obstructive lung disease: results from the United Kingdom biobank. J Am Med Dir Assoc 2014; 15 : 214–219.

Park SK, Richardson CR, Holleman RG, Larson JL . Frailty in people with COPD, using the National Health and Nutrition Evaluation Survey dataset (2003-2006). Heart Lung 2013; 42 : 163–170.

Fried LP, Ferrucci L, Darer J, Williamson JD, Anderson G . Untangling the concepts of disability, frailty and comorbidity: implications for improved targeting and care. J Gerontol A Biol Sci Med Sci 2004; 59 : 255–263.

Lahousse L, Maes B, Ziere G, Loth DW, Verlinden VJ, Zillikens MC et al. Adverse outcome of frailty in the elderly: the Rotterdam Study. Eur J Epidemiol 2014; 29 : 419–427.

Rockwood K, Howlett SE, MacKnight C, Beattie BL, Bergman H, Hebert R et al. Prevalence, attributes and outcomes of fitness and frailty in community-dwelling older adults: report from the Canadian study of health and aging. J Gerontol A Biol Sci Med Sci 2004; 59 : 1310–1317.

Rockwood K, Song X, MacKnight C, Bergman H, Hogan DB, McDowell I et al. A global clinical measure of fitness and frailty in elderly people. CMAJ 2005; 173 : 489–495.

Singanayagam A, Schembri S, Chalmers JD . Predictors or mortality in hospitalized adults with acute exacerbation of chronic obstructive pulmonary disease. Ann Am Thorac Soc 2013; 10 : 81–89.

Almagro P, Calbo E, Ochoa de Echaguen A, Barreiro B, Quintana S, Heredia JL et al. Mortality after hospitalization for COPD. Chest 2002; 121 : 1441–1448.

Groenewegen KH, Schols AM, Wouters EF . Mortality and mortality-related factors after hospitalization for acute exacerbation of COPD. Chest 2003; 124 : 459–467.

Soler-Cataluna JJ, Martinez-Garcia MA, Sanchez PR, Salcedo E, Navarro M, Ochando R . Severe acute exacerbations and mortality in patients with chronic obstructive pulmonary disease. Thorax 2005; 60 : 925–931.

Ball MM, Perkins MM, Whittington FJ, Connell BR, Hollingsworth C, King SV et al. Managing decline in assisted living: the key to aging in place. J Gerontol B Psychol Sci Soc Sci 2004; 59 : S202–S212.

Pynoos J, Caraviello R, Cicero C . Lifelong housing: the anchor in aging-friendly communities. Generations 2009; 33 : 26–32.

Zwerink M, Brusse-Keizer M, van der Valk PD, Zielhuis GA, Monninkhof EM, van der Palen J et al. Self management for patients with chronic obstructive pulmonary disease. Cochrane Database Syst Rev 2014; 3 : CD002990.

Bucknall CE, Miller G, Lloyd SM, Cleland J, McCluskey S, Cotton M et al. Glasgow supported self-management trial (GSuST) for patients with moderate to severe COPD: randomised controlled trial. BMJ 2012; 344 : e1060.

Fan VS, Gaziano JM, Lew R, Bourbeau J, Adams SG, Leatherman S et al. A comprehensive care management program to prevent chronic obstructive pulmonary disease hospitalizations: a randomized, controlled trial. Ann Intern Med 2012; 156 : 673–683.

Hull S, Mathur R, Lloyd-Owen S, Round T, Robson J . Improving outcomes for people with COPD by developing networks of general practices: evaluation of a quality improvement project in east London. NPJ Prim Care Respir Med 2014; 24 : 14082.

National Institute of Health and Clinical Excellence. Management of chronic obstructive pulmonary disease in adults in primary and secondary care. Updated 2010. NICE CG101. http://www.nice.org.uk/ . Accessed January 2015.

Gibson PG, Simpson JL . The overlap syndrome of asthma and COPD: what are its features and how important is it? Thorax 2009; 64 : 728–735.

Global INitiative for Asthma. Asthma, COPD and asthma-COPD overlap syndromes (ACOS). GINA 2014. http://www.ginasthma.org/documents/14 . Accessed January 2015.

Ries AL, Bauldoff GS, Carlin BW, Casaburi R, Emery CF, Mahler DA et al. Pulmonary Rehabilitation: Joint ACCP/AACVPR Evidence-Based Clinical Practice Guidelines. Chest 2007; 131 (5 Suppl): 4S–42S.

London Respiratory Team. Take a breath in, keep it in and now try to breathe again. NHS England, 2013. http://www.networks.nhs.uk/nhs-networks/london-respiratory-network . Accessed January 2015.

Guyatt G, Gutterman D, Baumann MH, Addrizzo-Harris D, Hylek EM, Phillips B et al. Grading strength of recommendations and quality of evidence in clinical guidelines: report from an American College of Chest Physicians task force. Chest 2006; 129 : 174–181.

Wilkinson TM, Donaldson GC, Hurst JR, Seemungal TA, Wedzicha JA . Early therapy improves outcomes of exacerbations of chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2004; 169 : 1298–1303.

Download references

Acknowledgements

The authors declare that no funding was received.

Author information

Authors and affiliations.

Agia Barbara Health Care Center, Heraklion, Crete, Greece

Ioanna Tsiligianni

Department of Thoracic Medicine, Clinic of Social and Family Medicine, University of Crete, Heraklion, Crete, Greece

Department of Medicine, College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada

Donna Goodridge

Division of Respirology, Critical Care and Sleep Medicine, University of Saskatchewan, Saskatoon, SK, Canada

Darcy Marciniuk

Centre for Primary Care and Public Health, Blizard Institute, Queen Mary University of London, London, UK

Respiratory Epidemiology and Clinical Research Unit, Montreal Chest Institute, McGill University Health Centre, Montréal, QC, Canada

Jean Bourbeau

You can also search for this author in PubMed   Google Scholar

Contributions

IT, DG and DM, JB, SH wrote the perspectives on case studies 1, 2, 3 and 4, respectively. The handling editor (Hilary Pinnock) collated and edited the individual sections.

Corresponding author

Correspondence to Jean Bourbeau .

Ethics declarations

Competing interests.

JB declares peer reviewed government grants (for conducting research in COPD self-management 'Living Well with COPD' and the longitudinal population-based Canadian Cohort Obstructive Lung Disease (CanCOLD) study) from Canadian Institute of Health Research Rx&D collaborative programme (Astra Zeneca, Boehringer- Ingelheim, GlaxoSmithKline, Merck, Nycomed, Novartis), Canadian Respiratory Research Network (CRRN), Respiratory Health Network of the FRQS and Research Institute of the MUHC. The remaining authors declare no conflict of interest.

Rights and permissions

This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

Reprints and permissions

About this article

Cite this article.

Tsiligianni, I., Goodridge, D., Marciniuk, D. et al. Four patients with a history of acute exacerbations of COPD: implementing the CHEST/Canadian Thoracic Society guidelines for preventing exacerbations. npj Prim Care Resp Med 25 , 15023 (2015). https://doi.org/10.1038/npjpcrm.2015.23

Download citation

Received : 22 February 2015

Accepted : 24 February 2015

Published : 07 May 2015

DOI : https://doi.org/10.1038/npjpcrm.2015.23

Share this article

Anyone you share the following link with will be able to read this content:

Sorry, a shareable link is not currently available for this article.

Provided by the Springer Nature SharedIt content-sharing initiative

Quick links

• Explore articles by subject
• Guide to authors
• Editorial policies

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Sign in for the best experience.

Your Connection to the Community of Chest Medicine

It's not too late to join CHEST Board Review

Live courses are under way, but you can still register and log on virtually to join sessions in progress—and get 12 months of free access to session recordings.

REGISTER NOW

BOARD REVIEW 2024 | Live sessions start August 22 for critical care medicine and August 25 for pulmonary medicine. REGISTER NOW »

Stay up-to-date with the latest in pulmonary, critical care, and sleep medicine

You should know.

Apply for Early Career Scholarship by August 30

Affirming Health Equity in Medicine in Support of Improving All Patient Outcomes

Advance your practice with hands-on simulation sessions, apps and pocus: overcoming credentialing challenges, changing the tumor board conversation: immunotherapy in resectable nsclc.

Free resources on RSV and the risk to patients

Understand the severity of respiratory syncytial virus (RSV), especially in patients who present with comorbidities such as COPD and asthma, in this interactive infographic and white paper.

EXPLORE THE INFOGRAPHIC

READ THE WHITE PAPER

Test your knowledge, prepare for exams, earn CME

Essential Bronchoscopy and EBUS Skills

Refine your skills in critical flexible bronchoscopy, September 5 to 7.

CHEST Curriculum Pathway for Lung Cancer: Treatment Highlights

Learn about lung cancer therapies and management, including biomarker testing, adjuvant and neoadjuvant therapies, and management of ground-glass nodules.

Ultrasonography: Essentials in Critical Care

Grow your competence in point-of-care ultrasonography with an emphasis on image acquisition and interpretation, September 12 to 13.

CCEeXAM Virtual Board Review

Hone your expertise of CCEeXAM concepts like image interpretation, clinical applications, stroke volume, and valve disorders.

Building Patient Trust e-Learning Modules

Learn how to quickly create rapport and build relationships with your patients.

Unveiling RSV

Gather samples, uncover transmission modes, and identify vulnerable parties to ensure a viral event doesn't occur.

SPECIAL OPPORTUNITY for new CHEST members when registering for simulation courses. LEARN MORE »

Join CHEST and take advantage of your membership benefits

CHEST brings together everything clinicians need for their careers, all in one place. A CHEST membership allows you to invest your time efficiently by offering comprehensive career resources for each stage of your career—from training through post-retirement.

BECOME A MEMBER CAREER CONNECTION

Already a member? SIGN IN »

Get to know John Coleman III, MD, FCCP

"The CHEST Networks offer great opportunities to meet and collaborate with those with the same clinical interests as you. Over the last several years, I have had the opportunity to not only collaborate on multiple review papers and guidelines but also have gained experience in presenting at international conferences."

Hear more from Dr. Coleman »

Raise awareness about your clinical trials with a critical audience

Industry companies are invited to share information about their clinical trials at CHEST 2024 in Boston. Recruit principal investigators/sites, reach clinicians who can help recruit or refer patients, and address the need for diversity in clinical trials by capturing the attention of our broad audience of pulmonary, critical care, and sleep medicine clinicians.

LEARN MORE & APPLY

200506-Diagnosis-and-management-of-COPD-a-case-study

• Nursing Times 116(6):36

• Swansea University

Abstract and Figures

Discover the world's research

• 25+ million members
• 160+ million publication pages
• 2.3+ billion citations
• Recruit researchers
• Join for free
• Login Email Tip: Most researchers use their institutional email address as their ResearchGate login Password Forgot password? Keep me logged in Log in or Continue with Google Welcome back! Please log in. Email · Hint Tip: Most researchers use their institutional email address as their ResearchGate login Password Forgot password? Keep me logged in Log in or Continue with Google No account? Sign up

COPD Case Study: Patient Diagnosis and Treatment (2024)

by John Landry, BS, RRT | Updated: May 16, 2024

Chronic obstructive pulmonary disease (COPD) is a progressive lung disease that affects millions of people around the world. It is primarily caused by smoking and is characterized by a persistent obstruction of airflow that worsens over time.

COPD can lead to a range of symptoms, including coughing, wheezing, shortness of breath, and chest tightness, which can significantly impact a person’s quality of life.

This case study will review the diagnosis and treatment of an adult patient who presented with signs and symptoms of this condition.

25+ RRT Cheat Sheets and Quizzes

Get access to 25+ premium quizzes, mini-courses, and downloadable cheat sheets for FREE.

COPD Clinical Scenario

A 56-year-old male patient is in the ER with increased work of breathing. He felt mildly short of breath after waking this morning but became extremely dyspneic after climbing a few flights of stairs. He is even too short of breath to finish full sentences. His wife is present in the room and revealed that the patient has a history of liver failure, is allergic to penicillin, and has a 15-pack-year smoking history. She also stated that he builds cabinets for a living and is constantly required to work around a lot of fine dust and debris.

Physical Findings

On physical examination, the patient showed the following signs and symptoms:

• His pupils are equal and reactive to light.
• He is alert and oriented.
• He is breathing through pursed lips.
• His trachea is positioned in the midline, and no jugular venous distention is present.

Vital Signs

• Heart rate: 92 beats/min
• Respiratory rate: 22 breaths/min

Chest Assessment

• He has a larger-than-normal anterior-posterior chest diameter.
• He demonstrates bilateral chest expansion.
• He demonstrates a prolonged expiratory phase and diminished breath sounds during auscultation.
• He is showing signs of subcostal retractions.
• Chest palpation reveals no tactile fremitus.
• Chest percussion reveals increased resonance.
• His abdomen is soft and tender.
• No distention is present.

Extremities

• His capillary refill time is two seconds.
• Digital clubbing is present in his fingertips.
• There are no signs of pedal edema.
• His skin appears to have a yellow tint.

Lab and Radiology Results

• ABG results: pH 7.35 mmHg, PaCO2 59 mmHg, HCO3 30 mEq/L, and PaO2 64 mmHg.
• Chest x-ray: Flat diaphragm, increased retrosternal space, dark lung fields, slight hypertrophy of the right ventricle, and a narrow heart.
• Blood work: RBC 6.5 mill/m3, Hb 19 g/100 mL, and Hct 57%.

Based on the information given, the patient likely has chronic obstructive pulmonary disease (COPD) .

The key findings that point to this diagnosis include:

• Barrel chest
• A long expiratory time
• Diminished breath sounds
• Use of accessory muscles while breathing
• Digital clubbing
• Pursed lip breathing
• History of smoking
• Exposure to dust from work

What Findings are Relevant to the Patient’s COPD Diagnosis?

The patient’s chest x-ray showed classic signs of chronic COPD, which include hyperexpansion, dark lung fields, and a narrow heart.

This patient does not have a history of cor pulmonale ; however, the findings revealed hypertrophy of the right ventricle. This is something that should be further investigated as right-sided heart failure is common in patients with COPD.

The lab values that suggest the patient has COPD include increased RBC, Hct, and Hb levels, which are signs of chronic hypoxemia.

Furthermore, the patient’s ABG results indicate COPD is present because the interpretation reveals compensated respiratory acidosis with mild hypoxemia. Compensated blood gases indicate an issue that has been present for an extended period of time.

What Tests Could Further Support This Diagnosis?

A series of pulmonary function tests (PFT) would be useful for assessing the patient’s lung volumes and capacities. This would help confirm the diagnosis of COPD and inform you of the severity.

Note: COPD patients typically have an FEV1/FVC ratio of < 70%, with an FEV1 that is < 80%.

The initial treatment for this patient should involve the administration of low-flow oxygen to treat or prevent hypoxemia .

It’s acceptable to start with a nasal cannula at 1-2 L/min. However, it’s often recommended to use an air-entrainment mask on COPD patients in order to provide an exact FiO2.

Either way, you should start with the lowest possible FiO2 that can maintain adequate oxygenation and titrate based on the patient’s response.

Example: Let’s say you start the patient with an FiO2 of 28% via air-entrainment mask but increase it to 32% due to no improvement. The SpO2 originally was 84% but now has decreased to 80%, and his retractions are worsening. This patient is sitting in the tripod position and continues to demonstrate pursed-lip breathing. Another blood gas was collected, and the results show a PaCO2 of 65 mmHg and a PaO2 of 59 mmHg.

What Do You Recommend?

The patient has an increased work of breathing, and their condition is clearly getting worse. The latest ABG results confirmed this with an increased PaCO2 and a PaO2 that is decreasing.

This indicates that the patient needs further assistance with both ventilation and oxygenation .

Note: In general, mechanical ventilation should be avoided in patients with COPD (if possible) because they are often difficult to wean from the machine.

Therefore, at this time, the most appropriate treatment method is noninvasive ventilation (e.g., BiPAP).

Initial BiPAP Settings

In general, the most commonly recommended initial BiPAP settings for an adult patient include this following:

• IPAP: 8–12 cmH2O
• EPAP: 5–8 cmH2O
• Rate: 10–12 breaths/min
• FiO2: Whatever they were previously on

For example, let’s say you initiate BiPAP with an IPAP of 10 cmH20, an EPAP of 5 cmH2O, a rate of 12, and an FiO2 of 32% (since that is what he was previously getting).

After 30 minutes on the machine, the physician requested another ABG to be drawn, which revealed acute respiratory acidosis with mild hypoxemia.

What Adjustments to BiPAP Settings Would You Recommend?

The latest ABG results indicate that two parameters must be corrected:

• Increased PaCO2
• Decreased PaO2

You can address the PaO2 by increasing either the FiO2 or EPAP setting. EPAP functions as PEEP, which is effective in increasing oxygenation.

The PaCO2 can be lowered by increasing the IPAP setting. By doing so, it helps to increase the patient’s tidal volume, which increased their expired CO2.

Note: In general, when making adjustments to a patient’s BiPAP settings, it’s acceptable to increase the pressure in increments of 2 cmH2O and the FiO2 setting in 5% increments.

Oxygenation

To improve the patient’s oxygenation , you can increase the EPAP setting to 7 cmH2O. This would decrease the pressure support by 2 cmH2O because it’s essentially the difference between the IPAP and EPAP.

Therefore, if you increase the EPAP, you must also increase the IPAP by the same amount to maintain the same pressure support level.

Ventilation

However, this patient also has an increased PaCO2 , which means that you must increase the IPAP setting to blow off more CO2. Therefore, you can adjust the pressure settings on the machine as follows:

• IPAP: 14 cmH2O
• EPAP: 7 cmH2O

After making these changes and performing an assessment , you can see that the patient’s condition is improving.

Two days later, the patient has been successfully weaned off the BiPAP machine and no longer needs oxygen support. He is now ready to be discharged.

The doctor wants you to recommend home therapy and treatment modalities that could benefit this patient.

What Home Therapy Would You Recommend?

You can recommend home oxygen therapy if the patient’s PaO2 drops below 55 mmHg or their SpO2 drops below 88% more than twice in a three-week period.

Remember: You must use a conservative approach when administering oxygen to a patient with COPD.

Pharmacology

You may also consider the following pharmacological agents:

• Short-acting bronchodilators (e.g., Albuterol)
• Long-acting bronchodilators (e.g., Formoterol)
• Anticholinergic agents (e.g., Ipratropium bromide)
• Inhaled corticosteroids (e.g., Budesonide)
• Methylxanthine agents (e.g., Theophylline)

In addition, education on smoking cessation is also important for patients who smoke. Nicotine replacement therapy may also be indicated.

In some cases, bronchial hygiene therapy should be recommended to help with secretion clearance (e.g., positive expiratory pressure (PEP) therapy).

It’s also important to instruct the patient to stay active, maintain a healthy diet, avoid infections, and get an annual flu vaccine. Lastly, some COPD patients may benefit from cardiopulmonary rehabilitation .

By taking all of these factors into consideration, you can better manage this patient’s COPD and improve their quality of life.

Final Thoughts

There are two key points to remember when treating a patient with COPD. First, you must always be mindful of the amount of oxygen being delivered to keep the FiO2 as low as possible.

Second, you should use noninvasive ventilation, if possible, before performing intubation and conventional mechanical ventilation . Too much oxygen can knock out the patient’s drive to breathe, and once intubated, these patients can be difficult to wean from the ventilator .

Furthermore, once the patient is ready to be discharged, you must ensure that you are sending them home with the proper medications and home treatments to avoid readmission.

Written by:

John Landry is a registered respiratory therapist from Memphis, TN, and has a bachelor's degree in kinesiology. He enjoys using evidence-based research to help others breathe easier and live a healthier life.

• Faarc, Kacmarek Robert PhD Rrt, et al. Egan’s Fundamentals of Respiratory Care. 12th ed., Mosby, 2020.
• Chang, David. Clinical Application of Mechanical Ventilation . 4th ed., Cengage Learning, 2013.
• Rrt, Cairo J. PhD. Pilbeam’s Mechanical Ventilation: Physiological and Clinical Applications. 7th ed., Mosby, 2019.
• Faarc, Gardenhire Douglas EdD Rrt-Nps. Rau’s Respiratory Care Pharmacology. 10th ed., Mosby, 2019.
• Faarc, Heuer Al PhD Mba Rrt Rpft. Wilkins’ Clinical Assessment in Respiratory Care. 8th ed., Mosby, 2017.
• Rrt, Des Terry Jardins MEd, and Burton George Md Facp Fccp Faarc. Clinical Manifestations and Assessment of Respiratory Disease. 8th ed., Mosby, 2019.

Recommended Reading

How to prepare for the clinical simulations exam (cse), faqs about the clinical simulation exam (cse), 7+ mistakes to avoid on the clinical simulation exam (cse), copd exacerbation: chronic obstructive pulmonary disease, epiglottitis scenario: clinical simulation exam (practice problem), guillain barré syndrome case study: clinical simulation scenario, drugs and medications to avoid if you have copd, the pros and cons of the zephyr valve procedure, the 50+ diseases to learn for the clinical sims exam (cse).

An official website of the United States government

The .gov means it’s official. Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

The site is secure. The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

• Publications
• Account settings

Preview improvements coming to the PMC website in October 2024. Learn More or Try it out now .

• Advanced Search
• Journal List
• Medicina (Kaunas)

Pulmonary Hypertension in COPD: A Case Study and Review of the Literature

Pulmonary hypertension (PH) is a frequently encountered complication of chronic obstructive pulmonary disease (COPD) and is associated with worsened clinical symptoms and prognosis. The prevalence of PH-COPD is not concretely established as classification criteria vary historically, but the presence of severe disease out of proportion to underlying COPD is relatively rare. Right heart catheterization, the gold standard in diagnosis of PH, is infrequently performed in COPD, and the overlap in the clinical symptoms of PH and COPD presents diagnostic challenges. Proven treatments are limited. Trials exploring the use of vasodilator therapy in this patient group generally demonstrate improvements in hemodynamics accompanied by worsening gas exchange without clearly demonstrated improvements in clinically meaningful outcomes. In-depth workup of underlying pulmonary hypertension and use of pulmonary vasodilator medications may be appropriate on an individual basis. We present a case study and a review and discussion of the pertinent literature on this topic.

1. Case Study

A man in his 60s with longstanding chronic obstructive pulmonary disease (COPD) presents himself as a new patient. He has a medical history of New York Heart Association (NYHA) Class I heart failure with a preserved ejection fraction of 60%, hypertension, hyperlipidemia, and a cerebrovascular accident six years ago with no residual deficits. He has smoked a pack of cigarettes a day from the age of 22 until quitting smoking at age 60 when he had a stroke. He indicates that he has been well-controlled on maintenance inhaler therapy, including a long-acting beta-agonist, long-acting muscarinic agent, and inhaled corticosteroid, and has never required oxygen therapy. His ability to perform daily activities is nevertheless somewhat limited by exertional dyspnea. His most recent pulmonary function testing indicates an forced expiratory volume in 1 second (FEV 1 ) of 1.98 L (63% of predicted) and a forced vital capacity (FVC) of 3.24 L (79% of predicted), both values obtained post-bronchodilator, with mildly elevated total lung capacity and residual volume suggesting hyperinflation and air trapping. The diffusion capacity of carbon monoxide on this testing is markedly reduced at 38% of predicted.

He reports a long period during which exacerbations of his COPD were infrequent, with none requiring hospitalization. However, about six months ago, he began to have gradually worsening exercise tolerance for which his inhalers seem to be largely ineffective and he has required two brief hospitalizations for acute exacerbations that were treated with nebulized bronchodilators, corticosteroids, and antibiotics. Concerned about progression of his congestive heart failure, his cardiologist sent him for a repeat transthoracic echocardiogram, which showed no appreciable change in the systolic or diastolic function of the left ventricle but showed new moderate dilation and moderate systolic dysfunction of the right ventricle. Right ventricular systolic pressure could not be measured due to lack of a tricuspid regurgitant jet. Concerned about his progressive symptoms, he presented himself to the outpatient pulmonary practice, where his resting oxygen saturation today is 91%. He has heard some mention of pulmonary hypertension and would like to discuss that topic as it pertains to him.

While the above case is hypothetical, it is typical of our clinical experience and introduces the theme of this review.

2. Discussion and Review of the Literature

2.1. overview, classification, and epidemiology.

Pulmonary hypertension (PH) has traditionally been defined by a mean pulmonary artery pressure of greater than 25 mmHg, though recent work to further classify the disease by the Sixth World Symposium on Pulmonary Hypertension has suggested a lower threshold of 20 mmHg along with pulmonary vascular resistance (PVR) of ≥3 Wood units for pre-capillary disease [ 1 ]. PH exists both as a discrete disease, as in pulmonary arterial hypertension (WHO Group I), or as a disease process attributable to other chronic diseases including chronic cardiac and pulmonary disease.

Efforts to classify pulmonary hypertension in COPD by a 2013 task force proposed to conceptually divide affected patients into two groups: (1) PH-COPD defined in a COPD patient by a mean pulmonary artery pressure (mPAP) ≥ 25 mmHg; and (2) severe PH-COPD, defined by the presence of mPAP ≥ 35 mmHg or ≥ 25 mmHg with the presence of a low cardiac index of <2.0 L/min/m 2 [ 2 ]. The task force suggested that the severe group of patients represented an important minority with disproportionately high rates of vascular remodeling and a loss of circulatory reserve that outpaced the loss of ventilatory reserve. The task force further suggested that this group should be the focus for studies that explore the use of pulmonary vasodilator medications in COPD patients. Indeed, available data suggest that severe PH-COPD represents a phenotype with markedly worsened exercise capacity and prognosis [ 3 ].

Prevalence estimates for PH in patients with COPD are not well-established, as right heart catherization is not routinely performed in this patient population and echocardiography is subject to diagnostic limitations. Estimation is further complicated by the use of a variety of different cutoff values to establish the presence of pulmonary hypertension in prior studies. A 1981 study of 175 patients with moderate-to-severe COPD who underwent right heart catheterization found that 35% had a mean pulmonary artery pressure of >20 mmHg [ 4 ]. At the higher end of estimation, a study of 120 patients with severe emphysema with an average FEV1 of 27% being evaluated for lung volume reduction surgery (LVRS) found that 90.8% of patients had mPAP > 20 mmHg [ 5 ]. Notably, no correlation was found between severity of emphysema and pulmonary artery pressure in this patient group. A study of 998 COPD inpatients admitted for respiratory failure with a mean FEV1 of 33% demonstrated that, while the mean mPAP was 20.3 mmHg, only 2.7% had severe PH, defined as an mPAP ≥ 40 mmHg, with only 1.1% of these patients having only COPD as an attributable cause of PH [ 6 ]. Overall, while elevated mean pulmonary artery pressures appear to be reasonably common in COPD, severe PH out of proportion to underlying lung disease is relatively rare.

2.2. Pathophysiology

In PH secondary to COPD, ongoing chronic hypoxic pulmonary vasoconstriction leads to changes that produce fixed remodeling of the pulmonary vasculature, namely fibromuscular intimal thickening and an increase in smooth muscle of the media of the pulmonary arterioles and arteries [ 7 ]. These same changes have also been noted in the pulmonary vasculature of smokers even without the presence of airflow obstruction [ 8 ]. Increased muscular hyperplasia of the microvasculature and decreases in alveolar capillary density have been found in severe PH-COPD patients, possibly representing a specific subgroup with regard to severity [ 9 ]. Loss of pulmonary vessels, long proposed as an underlying pathological feature of emphysema, may also occur and has been suggested as the key pathophysiological feature of a subtype of PH strongly associated with smoking and low diffusion capacity [ 10 , 11 ]. Increased levels of pulmonary vasoconstrictive mediators including endothelin-1 and decreased expression of endothelial nitric oxide synthase and prostacyclin synthase have also been observed in COPD compared to normal patients [ 12 , 13 , 14 , 15 ]. Genetic determinants also appear to play a role in the differential development of PH in patients with chronic lung disease [ 16 ].

The natural history of PH in COPD may begin with exercise-induced PH that precedes PH at rest. A study of 131 patients with COPD showed that in patients with mild-to-moderate COPD, progression of increases in right-sided pressures was slow, at roughly 0.4 mmHg per year, and that only about 25% of COPD patients with mild-to-moderate hypoxemia were found to have developed resting PH by six years. However, the presence of exercise-induced PH conferred a substantially greater risk of eventually developing resting PH [ 17 ].

These changes as a whole result in increased pulmonary vascular resistance and increased demand on the right ventricle. With progression of disease, the right ventricle may become overtly decompensated, resulting in cor pulmonale.

2.3. Diagnosis

Differentiating symptoms such as dyspnea on exertion or chest tightness as PH from underlying advanced COPD is challenging. The typical physical exam findings of precapillary pulmonary hypertension, such as a loud P2 and/or a holosystolic murmur of tricuspid regurgitation, may be less prominent in COPD patients or more difficult to appreciate due to distant heart sounds on exam. In addition, right heart pressures may not be elevated enough to produce these physical exam findings except during acute exacerbations when pulmonary pressures are demonstrably higher.

Spirometry values have not been shown to reliably correlate with the presence of underlying PH. Significant decrements in diffusion capacity for carbon monoxide (DLCO) may be suggestive of PH, though this is non-specific as this can also be observed in severe emphysema. However, the presence of a disproportionately reduced DLCO with respect to spirometric and radiographic changes of emphysema may be an indication of PH out-of-proportion to underlying COPD that would merit further investigation and treatment [ 6 ].

As discussed above, right heart catheterization, the gold standard for diagnosis of PH, is infrequently performed in patients with COPD unless specific cardiac indications exist, and there are currently no studies demonstrating its clinical usefulness in the routine evaluation of COPD.

Echocardiography represents a useful but limited modality to diagnose PH in the COPD population, with estimation of right ventricular systolic pressure (RSVP) from the velocity of tricuspid regurgitation considered to be a reliable method of detection when this signal is present [ 18 ]. However, in a study of 192 patients with advanced lung disease, including severe COPD, and 50 healthy controls, it was found that tricuspid regurgitation, which is critical for ultrasonographic assessment of PH, could not be assessed in 52% of patients [ 19 ]. In addition, parameters of right heart enlargement and dysfunction alone lacked sufficient specificity to reliably indicate PH in this patient population. Finally, the presence of gas trapping or hyperinflation may limit the echocardiographic technique and prevent accurate estimation of these values, with specificity for detection of PH of only 55% reported in one study of 374 patients with advanced lung disease, of whom over half (68%) had obstructive lung disease [ 20 ].

Imaging modalities such as computed tomography (CT) may offer promise for noninvasive diagnosis of pulmonary hypertension. A study of 60 patients with severe COPD (FEV1 of 27% ± 12%) found a linear correlation between the ratio of pulmonary artery to ascending aorta diameter with mean PA pressure, while such a correlation could not be observed using pulmonary artery systolic pressure (PASP) derived from echocardiography. However, the described area under the curve (AUC) of 0.83 is likely insufficient for truly accurate screening [ 21 ].

2.4. Treatment

Treatment options for COPD-PH remain limited outside of the routine inhaled medications to treat underlying COPD. Long-term oxygen therapy (LTOT) improves survival in COPD patients with hypoxemia and has also been shown to reduce mPAP over a six-month period, presumably by attenuating hypoxic pulmonary vasoconstriction [ 22 ]. However, LTOT does not have proven survival benefits in those with baseline oxygen saturations above the threshold of 89% [ 23 ].

Formal studies of vasodilator therapy in PH-COPD have been largely disappointing, with many demonstrating a measurable improvement in hemodynamics accompanied by worsening hypoxemia due to altered ventilation-perfusion matching, though some trials have shown no net change in oxygenation. Improvements in hemodynamics in these groups do not appear to consistently confer an improvement in symptoms, and some studies have noted worsening exercise tolerance with their use. Trials using pulmonary vasodilators in PH-COPD have also generally been limited by a short trial duration and small numbers of patients, limiting the strength of their conclusions.

Phosphodiesterase-5 inhibitors are often used as first-line therapy in pulmonary arterial hypertension and have been explored by a number of trials in PH-COPD patients. The Sildenafil and Pulmonary HypERtension in COPD (SPHERIC-1) trial was a double-blind, randomized, placebo-controlled trial of 28 patients with PH-COPD (FEV1 of 54% ± 22% in the sildenafil group) that followed patients for 16 weeks on sildenafil 20 mg three times daily [ 24 ]. Significant improvements in hemodynamics, including decreases in mPAP and pulmonary vascular resistance (PVR) and increases in cardiac index, were noted, and no detrimental effects on gas exchange or hypoxemia were found. In addition, BODE index and mMRC score improved, the latter by −0.51 in the experimental group. Notably, this study group had less severe baseline COPD than those typically studied in pulmonary vasodilator trials, with patients with FEV1 < 30% excluded, suggesting that ventilation-perfusion alterations may be subtler and better tolerated in less severe disease. A double-blind and placebo-controlled randomized controlled trial of 10 mg of daily tadalafil in 120 patients with COPD (mean FEV1 of 40%) and PH (measured by RVSP > 30 mmHg on echocardiography) described small improvements in right heart hemodynamics on echo but no improvement in exercise capacity or quality of life. There were no significant differences in SpO2 at the end of the 12-week study [ 25 ].

Endothelin receptor antagonists (ERAs) have also been studied with similar equivocal results. A study by Stolz et al. evaluated the use of bosentan, an endothelin receptor antagonist, in a double-blind randomized trial of 30 patients with severe or very severe COPD [ 26 ]. Of the 20 patients assigned to the bosentan group, six stopped the medication due to adverse side effects prior to the end of the trial. Six-minute walk time actually decreased in the bosentan group, as did mean PaO 2 . Notably, this study was limited by its lack of formal evaluation of the included patients for underlying PH prior to its initiation. A later randomized though not double-blinded trial of 32 COPD patients with confirmed PH (mPAP ≥ 25 mmHg on right heart catheterization) noted improvements in mPAP and PVR in the bosentan group as well as a slight improvement in BODE scores and did not show significant differences in PaO 2 at 18 months, suggesting greater promise for ERAs in a more select group of PH-COPD patients [ 27 ].

Inhaled pulmonary vasodilators have also been studied to a more limited degree in PH-COPD patients. Wang et al. studied the efficacy and safety of inhaled iloprost, a synthetic prostacyclin, in 67 PH-COPD patients, 37 of whom had severe disease by the above criteria and found an average decrease in mPAP of −2.1 mmHg and increase in cardiac output of 0.4 L/min after a single 20 µg nebulized dose without significant changes in PaO 2 or PaCO 2 [ 28 ]. A 2016 study of inhaled treprostinil in a small group of nine PH-COPD patients also found no change in oxygenation based on arterial blood gas but unexpectedly showed decreases in FEV1, FVC, and DLCO [ 29 ].

Real-world use of pulmonary vasodilator medications in PH-COPD has also met with mixed results. Of the 101 patients included in the ASPIRE registry, 43 patients with severe PH-COPD were treated compassionately, mostly with PDE-5 inhibitors, for a duration of at least three months. Survival among these patients was only 72% at one year, not significantly different from the 16 untreated patients despite the treatment group having significantly worse baseline hemodynamics. Of these 43 patients, eight of them showed evident clinical improvement and did show an improvement in survival, suggesting as yet undetermined factors in their disease that rendered vasodilator therapy more beneficial [ 3 ]. Of particular interest, the study did not note significant perturbations in oxygen saturation in patients on vasodilator therapy, though the authors caution that the study was not designed to assess this.

2.5. Prognosis

The negative prognostic implications of an elevated mean pulmonary artery pressure in COPD are well-understood. A 1981 study by Weitzenblum et al. of 175 patients with moderate-to-severe COPD found that survival rates were markedly lower in those that had mPAP above 20 mmHg at four and seven year follow-up, with survival rates at four years of 71.8% in those with mPAP < 20 mmHg and 49.4% in those above 20 mmHg [ 4 ]. In this study, mPAP was as strong a factor for predicting survival as PaO 2 , PaCO 2 , and FEV1. More recently, the ASPIRE registry found a 3-year survival of only 33% in those with severe PH-COPD, defined as mPAP of 40 mmHg or greater [ 3 ].

Many recent studies have suggested that the burden of mortality in PH-COPD is at least as high if not higher than that of idiopathic PAH (IPAH), itself a disease with notoriously high mortality rates. A 2015 study of 1472 PH patients included in the COMPERA registry revealed a 3-year survival rate of 70.7% in IPAH but only 58.8% in PH-COPD [ 30 ]. Rates of mortality were noted to be lower at one, two, and three years in PH-COPD compared to PH-ILD. A 2019 study comparing 51 patients with PH due to chronic lung disease to 83 patients with IPAH demonstrated that the former patient group had equally poor outcomes despite lower mean pulmonary artery pressure and pulmonary vascular resistance [ 31 ]. A PVR of seven Wood units or greater was noted to confer a 3-fold higher risk of mortality and was more strongly associated with mortality than mPAP. Similar to the COMPERA registry above, 5-year survival was significantly lower amongst those with PH-ILD compared to PH-COPD.

In addition to markedly worsened overall survival, the presence of pulmonary artery enlargement on CT, implicative of higher mean pulmonary artery pressure, is associated with increased risk of severe COPD exacerbations requiring hospitalization [ 32 ].

3. Return to the Case Study

Given the presence of hypoxemia and severe dyspnea out of proportion to the spirometric defects present in the patient, as well as the severe defect in diffusion capacity, the possibility of significant pulmonary hypertension secondary to COPD is felt to be a reasonable diagnostic possibility. A repeat non-contrast CT scan of the chest that is obtained to evaluate for concurrent interstitial lung disease demonstrates emphysematous changes more prominent in the upper lobes; enlargement of the main pulmonary artery is also noted compared to on the last CT scan performed roughly one year prior for lung cancer screening.

A discussion between the patient, pulmonologist, and cardiologist is held regarding the risks and benefits of right heart catheterization, given that echocardiography suggested but could not estimate right heart pressures and a repeat CT scan demonstrated an interval increase in the width of the main pulmonary artery.

The patient consents to undergo right heart catheterization, which reveals a mean pulmonary artery pressure of 37 mmHg and a pulmonary capillary wedge pressure of 13 mmHg. Vasoreactivity testing was not performed. Alternative etiologies to explain this underlying pulmonary hypertension are discussed. The patient reveals he underwent polysomnography roughly two and a half years prior that did not demonstrate obstructive sleep apnea. A repeat STOP-BANG screening in clinic results in a score of 2, and the decision is made not to repeat sleep testing. A ventilation-perfusion scan to rule out chronic thromboembolic pulmonary disease (CTEPH) is considered, but the patient denies any history of thromboembolic disease.

Having effectively ruled out other likely contributing factors, the elevated right heart pressures are attributed to the known underlying lung disease and the diagnosis of severe PH-COPD is made. A trial of sildenafil (20 mg) three times daily is begun; after four weeks, the patient reports a mild improvement in exertional dyspnea but is noted to have a slight worsening in baseline oxygen saturation to 89%. Despite this change, he opts to continue the medication in light of his perception of a slight increase in exercise tolerance. In light of this worsening in baseline oxygen saturation, he is started on 2 L/min supplemental oxygen. He is offered referral to pulmonary rehabilitation but declines.

After six months, he returns to clinic and reports that both his ability to perform activities of daily living and his exertional dyspnea have not changed, though the purchase of a portable oxygen concentrator has made leaving his home easier. His oxygen saturation is 93% on 2 L/min in the office. A repeat echocardiogram is ordered that demonstrates continued moderate right ventricular systolic dysfunction, but mild dilatation of the ventricle compared to the moderate dilatation shown in the previous study; a tricuspid regurgitant jet again cannot be measured. A repeat six-minute walk distance is shown to be 37% of predicted from the 34% six months prior. In light of the patient’s lack of significant clinical improvement and his concerns of the cost of ongoing sildenafil therapy, the medication is stopped, and he is continued on his inhaled regimen for COPD and supplemental oxygen.

No external funding was provided for this review.

Conflicts of Interest

None of the authors disclose any conflicts of interest, either real or perceived.