Summary

Description
Chronic obstructive pulmonary disease (COPD) is a disease state characterized by airflow limitation that is not fully reversible Airflow limitation is usually both progressive and associated with an abnormal pulmonary inflammatory response to noxious particles or gases Management plan focuses on four main components: assess and monitor; reduce risk factors; manage stable COPD; and manage exacerbations
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Synonyms
COPD Chronic obstructive airway disease COAD Chronic irreversible airway disease Chronic airflow limitation COLD Chronic obstructive lung disease
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Urgent action
If the patient is severely tachypneic, dyspneic, cyanotic (and not polycythemic), or has a significant change in mental status consistent with hypoxia or hypercapnia, then transfer to emergency room for evaluation and treatment of possible respiratory failure.
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Key points
Irreversible airway obstruction COPD presents a spectrum of chronic bronchitis and emphysema Only smoking cessation and home oxygen therapy (if indicated) have been shown to improve survival Pharmaceutical management and pulmonary rehabilitation improve symptoms and decrease the number of exacerbations and hospitalizations

Background
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Cardinal features
Airflow limitation that is not fully reversible (asthma is typically reversible)

Airflow limitation is progressive and caused by a mixture of airway disease and parenchymal destruction Airflow limitation is associated with an abnormal pulmonary inflammatory response to noxious particles or gases Chronic inflammation causes remodeling and narrowing of small airways Decreased lung elastic recoil Expiratory small airway collapse Chronic cough and sputum production may or may not be present
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Causes
Common causes
Risk/causative factors for chronic obstructive pulmonary disease (COPD) can be separated into two categories: host factors and exposures. The disease state results from interactions between these factors. Host factors: Genetic predisposition Airway hyperresponsiveness Retarded lung growth (history of shortened gestational age, low birthweight, and factors during childhood)

Exposures (total burden of inhaled particles) - exposures are additive: Tobacco smoke Occupational dusts and chemicals (vapors, irritants, and fumes) Environmental exposure to dusts, particles, and chemicals

Rare causes
Hereditary deficiency of alpha-1-antitrypsin glycoprotein.
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Epidemiology
Incidence and prevalence
This disease tends to be significantly underdiagnosed; it is possible that only half of those with COPD are included in prevalence data.

Incidence
New diagnosis of COPD is more common in women, owing to diagnosis in aging women who have smoked since their teenage years.

Prevalence
Approx. 6000/100,000.

Demographics

Age
COPD prevalence increases with increasing age; number of pack-years exposure to cigarette smoke is critical.

Gender
In developed countries, men have been affected more than women, probably because of greater exposure to cigarette smoke in older men. This gap is narrowing Recent US population studies show almost equal prevalence of COPD, owing to women's smoking habits over the past 50 years

Genetics
Genetic predisposition to alpha-1-antitrypsin deficiency.

Geography
No evidence; however, long-term exposure to air pollutants has been associated with COPD.

Socioeconomic status
There is no evidence that socioeconomic status is directly linked to COPD; however, people of low socioeconomic status are more likely to smoke cigarettes, are more likely to have low birthweight babies, and are more likely to be exposed to indoor pollutants from cooking and heating fuels that can increase risk of COPD. More research is needed in this regard.

Clinical presentation
Symptoms

Chronic cough: usually the first sign to appear (however, some patients may have significant airflow limitation without cough or may deny cough) Any pattern of chronic sputum production Dyspnea, breathlessness (usually the reason patients seek care) that is progressive, persistent, worse on exercise, and worse during respiratory infections Chest tightness (nonspecific and variable) Anxiety

Signs

Wheezing (nonspecific and variable) Decreased breath sounds (quietest over bullae) Cyanosis is a late sign in the absence of polycythemia Signs of lung hyperinflation Decreased measurements of airflow on spirometry
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Associated disorders

Cor pulmonale and right heart failure may result from chronic pulmonary vasoconstriction caused by chronic hypoxemia and significantly increased right ventricular afterload Cardiac arrhythmias, such as atrial fibrillation, may be associated with chronic obstructive pulmonary disease (COPD) Other disorders related to smoking such as ischemic heart disease Anxiety and depression are common in patients with COPD Other comorbidities include bronchial carcinoma, sleep apnea, and heart failure
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Differential diagnosis
Note that the new GOLD guidelines no longer differentiate between emphysema and chronic bronchitis. Asthma Bronchiectasis Pulmonary tuberculosis
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Workup
Diagnostic decision
According to the GOLD practice guidelines, COPD is now staged. Diagnosis may be made at any stage of the illness. Stage 0 - at risk:

Spirometry still normal Typically, chronic cough and sputum production

Stage I - mild COPD:

Forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) <70%, but FEV1 >80% of predicted value Usually, but not always, chronic cough and sputum production Patient may not be aware that lung function is abnormal

Stage II - moderate COPD:

FEV1/FVC <70% with FEV1 between 50% and 80% of predicted value Typically, the stage at which patients first seek medical attention because of dyspnea developing on exertion or an exacerbation of their disease

Stage III - severe COPD:

FEV1/FVC <70% with FEV1 between 30% and 50% of predicted value Increased shortness of breath and repeated exacerbations, impacting on the quality of life

Stage IV - very severe COPD:

FEV1/FVC <70% with FEV1 <30% of predicted value OR presence of chronic respiratory failure or clinical signs of right heart failure due to cor pulmonale Respiratory failure: pO2 <60mmHg with or without pCO2 >50mmHg while breathing air (21% oxygen) at sea level Patients may have severe COPD even if FEV1 is >30% of predicted value when complications of right heart failure or respiratory failure are present

Don't miss!
Cigarette smoking is the single most important risk factor for COPD. Be sure to inquire about smoking habits and history in a nonthreatening manner for each patient you see The National Lung Health Education Program strongly recommends the use of office spirometry in primary care to diagnose and monitor COPD patients Signs suggesting coexisting bronchial carcinoma Hypoxia - oxygen supplementation for hypoxic patients has been demonstrated to improve survival and function Nocturnal hypoxemia - if patients demonstrate a significant fall in oxygen levels (O2 saturation falls below 88% or pO2 of 55mmHg), nocturnal treatment with oxygen is warranted Carbon dioxide retention and hypercapnia - sample ABGs 20-30min after starting oxygen therapy to detect CO2 retention. It is difficult to predict who will retain CO2

Questions to ask Presenting condition

What is bothering you the most right now? The patient may indicate that they have shortness of breath, cough, and/or cannot perform activities of daily living. For symptom of greatest concern, rate on 1-10 scale (1 is normal, 10 is worst). This provides an objective way to compare symptomatic episodes over time How far can you walk (how many stairs) before you become short of breath? This is a good question to assess functional impairment secondary to lung disease Has a health care provider ever told you that you have asthma? Do you have or have you ever had allergies, sinusitis, or nasal polyps? These questions help to differentiate COPD from asthma How did your chief complaint start and how has it progressed to today? Asthma tends to have asymptomatic periods with acute exacerbations that may come on quickly. It also tends to occur in younger patients. COPD is a slow, steady deterioration of lung function Have you ever been admitted to hospital for lung disease? The severity of disease may be indicated by the number of hospital admissions (postdiagnosis)

Do you cough every day? Chronic cough is suggestive of COPD Do you find you have to clear your throat more often than other people? Do you cough up phlegm? What does it look like? Many people deny a chronic cough because they are afraid to admit it, but will state they clear their throat or produce sputum (a good strategy to get correct information from a reluctant patient) Have you had fever, chills, change in color of sputum, chest pain when you breathe, unusual fatigue, sore throat, or change in cough? Evaluates potential for infection

Contributory or predisposing factors

Do you smoke cigarettes? Have you ever smoked cigarettes? If yes, how many packs and for how many years? If yes, when did you first start smoking? If yes, have you ever tried to quit? If I helped you, would you be willing to try to quit? Cigarette smoking is the single most important risk factor for COPD Have you lived in the same dwelling as someone (or more than one person) who smoked cigarettes? How much did they smoke and for how many years were you exposed? At what age were you exposed? Passive exposure to cigarette smoke increases the lungs' total burden of inhaled particles and gases What type of work have you done during your life? Have you been in environments in which you might have inhaled dusts or chemicals at work? How do you cook at home? How do you heat your house? Where do you live? Evaluates potential occupational and environmental exposures Did you have a lot of respiratory infections as a child? Evaluates potential for underlying lung underdevelopment Do you have any other respiratory disease(s)? Do you have any heart disease? Other diseases can contribute to dyspnea, limitation of activities, and loss of work

Family history
Is there a history in your family of COPD, emphysema, asthma, or chronic bronchitis? Think aboutalpha-1-antitrypsin deficiency if a patient has symptoms before age 40.

Examination
Note that a physical examination is important, but rarely diagnostic in COPD. Record the temperature, heart rate, respiratory rate: fever, tachycardia and tachypnea are consistent with infection and hypoxia Perform a general inspection: cyanosis is a late sign in the absence of polycythemia, correlating to a pulse oximetry reading <70% if hemoglobin level is normal. Need to observe for clubbing Inspect for objective signs of respiratory distress: look for nasal flaring, pursed-lip breathing, accessory muscle use (particularly scalene and sternocleidomastoid) and retractions Examine for signs of chronic hyperinflation: inspect for relatively horizontal ribs, barrel-shaped chest, and protruding abdomen. Diaphragms may be flattened on percussion Percuss chest: identify any hyperresonance, which reflects air trapping, or dullness resulting from underlying consolidation (tumor or pneumonia)

Auscultation: often nonspecific; absence of vesicular lung sounds is common as air trapping becomes more prominent. Wheeze and prolonged expiration are common Examine sputum if patient is able to provide specimen: clear, white, gray are normal for COPD Examine for dependent edema and jugular venous distension: these could signal coexistingcongestive heart failure or cor pulmonale

Summary of tests
Office spirometry should be done in any patient suspected of having COPD, as recommended by theNational Lung Health Education Program and GOLD practice guidelines. Screening spirometry should be done on all smokers in order to record lung function over time and detect deterioration before symptoms appear. Abnormalities in initial office spirometry should be followed up with formal diagnostic spirometry from a pulmonary function laboratory Chest X-ray may show features of COPD and may rule out alternative diagnoses Bronchodilator reversibility testing is performed in a pulmonary function testing laboratory Arterial blood gas measurement indicates the presence of hypoxia or hypercapnia Alpha-1-antitrypsin deficiency screening is normally performed by a pulmonologist in patients under 40 years with COPD symptoms and a suggestive family history; family counseling and follow-up may be needed. The test is also recommended for patients with predominantly lower lobe emphysema and in those patients who are non-smokers, but have emphysema. The testing does not need to be done by a pulmonologist, but if abnormal, a referral should be made

Order of tests
Office spirometry Chest X-ray Bronchodilator reversibility testing Arterial blood gas measurement Alpha-1-antitrypsin deficiency screening

Clinical pearls

Children exposed to cigarette smoke have more respiratory symptoms and diseases than do children of nonsmokers COPD is not only the fourth leading cause of death in the US, but it is also the fastest rising one Normal adult nonsmokers lose about 30mL of their FEV1/year; patients with COPD lose about 90mL/year Persistent production of purulent sputum suggests the diagnosis of bronchiectasis. Supplemental oxygen prolongs the life of patients who have pO2 values <59mmHg or hematocrit >55% Screen for nocturnal hypoxemia in patients who are obese, are carbon dioxide retainers, or show evidence of right-sided heart failure despite pO2 values >59mmHg

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Consider consult

Consider referral if there is uncertainty of the diagnosis, particularly between asthma and COPD because each is treated differently Consider referral if recurrent infections do not respond to traditional antibiotic therapy

Treatment
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Goals

The patient will learn the nature of the disease, risk factors that could cause disease progression, and the role of the patient and that of the health care providers in managing the disease and achieving optimal outcomes Prevent disease progression Work with the patient to optimize day-to-day functioning by reducing symptoms and improving exercise tolerance Develop an open, nonthreatening, nonjudgmental relationship with the patient Reduce the number of exacerbations and superimposed infections Prevent and treat complications Support the patient through smoking cessation Reduce mortality Reverse hypoxia and maintain adequate oxygenation (pO2 >60mmHg or SaO2 >88%)
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Immediate action
Referral is required if patient goes into respiratory failure and requires mechanical ventilatory support.
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Therapeutic options
Summary of therapies
According to the GOLD practice guidelines, treatment includes three aspects:

Reducing risk factors

Managing stable chronic obstructive pulmonary disease (COPD) Managing COPD exacerbations

Reduce risk factors - lifestyle changes:

Smoking cessation is the single most effective way to reduce the risk of developing COPD and stopping its progression in most people; all tobacco users should be offered help to quit during every visit with a health care provider Reduce the burden of inhaled particles and gases through occupational exposure and indoor and outdoor air pollution

Manage stable COPD:

Health education plays a key role in improving health status Pharmacotherapy has no effect on the long-term decline in lung function; it is used to reduce symptoms and/or decrease or prevent complications Bronchodilator medications (ipratropium and beta-2 agonists) are the cornerstone of symptom management in COPD. A long-acting anticholinergic, tiotropium, has recently become available. Theophylline is effective in COPD, but because of its potential toxicity, inhaled bronchodilators are preferred when available There should be a stepwise increase in treatment, depending on the severity of the disease. Regular treatment should be maintained at a constant level for long periods unless significant side-effects occur or the disease worsens Mild COPD: for disease management, one of the short-acting inhaled bronchodilator agents should be used as needed for symptom control Moderate, severe, and very severe COPD: in the absence of symptom control with as-needed short-acting bronchodilators, the addition of regular bronchodilator treatment is recommended. As a choice of regular treatment, long-acting inhaled bronchodilators are more effective and convenient than shortacting bronchodilators, but more expensive. Regular therapy with either a shortor long-acting bronchodilator may be combined with as-needed use of a shortacting bronchodilator Corticosteroids are not recommended in mild and moderate COPD Severe and very severe COPD: regular treatment with inhaled corticosteroids is appropriate for symptomatic patients with FEV1 <50% predicted and repeated exacerbations. A combined inhaled glucocorticosteroid with long-acting beta-2 agonist (fluticasone proprionate plus salmeterol) is available in three strengths Avoid chronic treatment with systemic corticosteroids because the risks of longterm treatment outweigh benefits in this patient population Pulmonary rehabilitation programs improve exercise tolerance, and reduce dyspnea and fatigue

Oxygen therapy >15h/day for COPD patients in chronic respiratory failure can increase survival. The goal of oxygen therapy should be oxygen saturation of 8890%

Manage exacerbations:

Bronchodilator therapy (ipratropium, combined albuterol and ipratropium, and beta-2 agonists) For exacerbations at home: increase the dose and/or frequency of bronchodilator therapy from baseline. If not already used, add additional bronchodilator from a different class, i.e. if on beta-agonist, add an anticholinergic and vice-versa until symptoms improve. In more severe cases, high-dose nebulizer therapy may be given on an as-needed basis for several days In hospital: short-acting inhaled beta-2 agonist combined with anticholinergic drugs are preferred. These are usually given every 4h with an every-2h asneeded dose Systemic corticosteroids are beneficial in management of acute exacerbations; used in conjunction with bronchodilators Antibiotics should only be used when there is increased sputum production and purulence; the choice of antibiotic agent should be based on local patterns of sensitivity and culture results. For uncomplicated patients, stage II, or stable stage III patients, first-line antibiotics are the less expensive drugs, amoxicillin,doxycycline, or trimethoprim-sulfamethoxazole. For complicated patients, stage III with frequent exacerbations, or stage IV patients, more broad-spectrum antibiotics such as levofloxacin or amoxicillin-clavulanic acid are commonly used Hospital admission for management if exacerbation is severe Controlled oxygen therapy Ventilator support for complete respiratory failure is a therapy instituted in the acute hospital setting

Surgical interventions (selected cases):

Lung volume reduction surgery may improve lung capacity and function in patients with emphysema, as documented by pulmonary function testing and chest tomography. The surgery is reserved for patients with severe emphysema. The long-term consequences of the operation are not yet known Lung transplantation is reserved for younger patients with life expectancy of <2 years. The survival advantage of lung transplantation for COPD has not been established, but for patients in whom the procedure is successful, marked improvement in the quality of life can be expected

Order of therapies

Lifestyle changes

Ipratropium Beta-2 agonists Tiotropium Inhaled corticosteroids Corticosteroids Pulmonary rehabilitation Oxygen therapy Amoxicillin Doxycycline Trimethoprim-sulfamethoxazole Levofloxacin

Efficacy of therapies

Therapies for COPD do not reverse the pathology of the disease; they are used to treat symptoms only Efficacy of therapy is based on patient reports of symptom improvement; a number of health scoring forms may be used, or a simple interview

Summary of evidence Evidence Short-acting anticholinergics are effective for the short-term symptomatic management of stable COPD.

Several randomized controlled trials (RCTs) have found that anticholinergics are significantly more effective than placebo for the improvement of forced expiratory volume in one second (FEV1) and exercise capacity [[1]]Level A

Differences in the benefit of ipratropium and short-acting beta-2 agonists in the management of stable COPD appear small.

In stable COPD, the choice between beta-2 agonist, anticholinergic, theophylline, or combination therapy depends on availability and individual response in terms of symptom relief and side-effects [[21]]Level C One systematic review studying treatment for exacerbations, found no significant difference in changes in FEV1 between patients treated with short-acting beta-2 agonists and ipratropium whereas another systematic review found a small advantage in the regular long term use of ipratropium alone over a beta-2 agonist alone in terms of improving lung function, symptoms and exercise tolerance in the management of stable COPD [[2], [3]]Level A

Short-term treatment with ipratropium plus a short-acting or longacting inhaled beta-2 agonist in stable COPD appears to have some benefit over the individual bronchodilator therapies but more research is necessary.

There is evidence that regular inhalations of a combination of ipratropium and a short-acting beta-2 agonist is associated with a significant reduction in exacerbations of COPD or with improvements in lung function, symptoms and exercise tolerance vs regular short-acting-beta-2 agonist alone [[3], [5]]Level A A combination therapy of ipratropium bromide plus a long-acting beta-2 agonist may have some benefit in terms of post-bronchodilator lung function, supplemental short-acting beta-2 agonist use and health-related quality-of-life score compared with salmeterol alone [[4]]Level A An RCT found that the combined treatment over 12 weeks was associated with a significant improvement in FEV1 compared with salmetrol alone although there was no difference in symptoms between the two treatment options [[6], [7]]Level A

There is little to support short-term treatment with combined ipratropium plus a short-acting beta-2 agonist in the treatment of exacerbations of COPD compared to the individual therapies taken alone.

A systematic review studying treatment for exacerbations, failed to find any significant increase in bronchodilation associated with combining ipratropium with a short-acting beta-2 agonist compared with either individual therapy taken alone [[2]]Level

There is evidence that short-acting beta-2 agonists in COPD improves lung function compared with placebo.

Bronchodilators are prescribed on an as-needed or on a regular basis to prevent or reduce symptoms [[21]]Level C Regular use of short-acting beta-2 agonists, for at least 1 week, in patients with stable COPD has been found to result in a small but significant improvement in FEV1 and breathlessness score compared to placebo [[8]]Level A

Long-acting beta-2 agonists improve lung function and reduce exacerbations in COPD compared with placebo.

In people with COPD with poor reversibility to short-acting bronchodilators, salmeterol 50 mcg taken twice daily has been shown to produce modest increases in lung function and an associated reduction in exacerbations [[9]]Level A One systematic review found that long-acting beta-2 agonists significantly reduced COPD exacerbations and improved health-related quality-of-life compared with placebo at 12-52 weeks whereas another systematic review

found little difference between ipratropium and salmeterol in terms of symptoms and exercise tolerance [[5], [4]]Level A

Long-acting anticholinergics have been found to significantly reduce the number of exacerbations in people with moderate to severe stable COPD.

A systematic review found that inhaled tiotropium, compared to placebo or ipratropium, significantly reduced the risk of exacerbation and related hospitalization, and improved health-related quality-of-life and symptom scores among patients with moderate and severe disease. There was however no statistically significant difference in outcome when tiotropium was compared with long-acting beta-2-agonists [[12]]Level A

Oral corticosteroids are effective in the management of acute exacerbations of COPD but this is at the expense of an increased risk of adverse drug reaction. Long-term therapy is associated with serious adverse effects.

A systematic review found that treatment of acute exacerbations of COPD with systemic steroids produced a significant reduction in treatment failure but no significant difference in mortality compared with placebo [[13]]Level A There is some evidence that higher doses (30 mg prednisolone) improve lung function over a short period [[14]]Level A Chronic treatment with systemic glucocorticoids should be avoided because of an unfavorable benefit-to-risk ratio [[21]]Level C

Evidence concerning inhaled corticosteroids has been conflicting. Any benefit of long-term therapy needs to be balanced with the possibility that it may predispose to adverse effects.

The addition of regular inhaled corticosteroids to bronchodilator treatment is appropriate for symptomatic COPD patients with an FEV1 <50% predicted (stage III: severe COPD and stage IV: very severe COPD) and repeated exacerbations [[21]]Level C There is evidence to suggest that long-term treatment with inhaled corticosteroids may have some beneficial effect on lung function and reduce exacerbation rates in selected patients, but short-term treatment does not. The evidence from the different trials is not entirely consistent [[1], [5], [15], [16], [19],[20]]Level A

There is limited evidence for the benefit of the combined long-acting beta-2 agonist plus corticosteroid inhaler.

A systematic review found treatment with corticosteroid/long-acting beta-2 agonist in a combined inhaler was associated with a reduction in exacerbation rate and improvement in quality of life and lung function compared with placebo [[11]]Level A

Pulmonary rehabilitation significantly improves mortality rates, along with various other outcome measures in people with COPD.

Patients with COPD at all stages benefit from exercise training programs. Sustained benefit may be achieved even after a single pulmonary rehabilitation program [[5]]Level C Two systematic reviews have found evidence that the use of pulmonary rehabilitation in the treatment of COPD significantly improves a number of important outcomes, including mortality, hospital admissions, health-related quality of life, and exercise capacity, when compared to usual treatment [[25], [26]] Level A

Long-term oxygen therapy improves survival in a selected group of patients with COPD.

Long-term administration of oxygen (>15h per day) has been shown to improve survival in patients with chronic respiratory failure. Oxygen may improve exercise capacity, lung mechanics, mental state, hemodynamics, and hematologic features [[21]]Level C A systematic review included RCTs that found that long-term oxygen therapy improved survival in a selected group of patients with COPD and severe hypoxemia [[23]]Level A However people with COPD with mild-moderate hypoxemia or those with desaturation during the night receiving nocturnal oxygen or long-term oxygen do not appear to have any such benefit in survival compared with no oxygen therapy [[23]]Level A

Non-invasive positive-pressure ventilation is a useful therapy in respiratory failure, especially if given early.

There is evidence to suggest that noninvasive positive-pressure ventilation as a first-line treatment and an adjunct therapy to usual therapy is of benefit in the management of respiratory failure secondary to acute exacerbations of COPD. Early use in respiratory failure, before the onset of severe acidosis, may reduce the likelihood of endotracheal intubation, treatment failure, and death. However, it is also associated with numerious adverse events, and staff must be fully trained in its use. There must be 24-hour cover by an appropriately qualified member of the medical team [[24]]Level A

Action plans for COPD can help patients to recognize and respond to acute exacerbations.

There is evidence that action plans help people with COPD to recognize and react appropriately to acute exacerbations. However, it is not clear whether there is a significant effect on morbidity or mortality [[27]]Level A

Clinical pearls

For patients with COPD, smoking cessation is associated with a small increase in FEV1 and fall in yearly loss of FEV1 back toward the rate of loss of normal nonsmokers. Pharmacologic interventions do not change the rate of loss of FEV1 Chronic, outpatient oxygen supplementation for patients who meet the criteria described above is the only therapeutic intervention conclusively demonstrated to improve survival
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Never
It was commonly taught that too much oxygen could cause a COPD patient to stop breathing by knocking out the patient's 'hypoxic drive' for respiration. In reality, administering oxygen relieves hypoxic pulmonary vasoconstriction, thereby increasing dead space and resulting in hypercapnia. The hypercapnia is not caused by hypoventilation resulting from a blunting of the so-called 'hypoxic drive'. It is far more dangerous to withhold oxygen from COPD patients. The goal of oxygen therapy should be oxygen saturation of 88-90%.
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Management in special circumstances

Coexisting disease
Management of COPD may be complicated by coexistent congestive heart failure Chronic hypoxia and hypercapnia promote sodium absorption and edema by decreasing glomerular filtration, decreasing filtration of sodium, and the retention of bicarbonate Chronic hypoxia and hypercapnia may increase the risk of digoxin toxicity If peripheral edema is due to cor pulmonale, it is less responsive to diuresis. Care must be taken to avoid intravascular volume depletion Left ventricular function can be impacted in COPD by impaired left ventricular filling owing to exaggerated negative swings in intrathoracic pressure and diastolic interdependence of the ventricles Pulmonary edema more quickly leads to hypoxia and hypercarbia

Coexisting medication
In rare cases, sympathomimetic therapy for COPD may increase heart rate; this could reduce cardiac output in patients with heart disease Beta-blockers, which are recommended to reduce myocardial oxygen demand following myocardial infarction (or to treat hypertension), should be carefully evaluated for use in COPD patients because of the risk of bronchospasm. In the case of a previous myocardial infarction, the use of beta-blockers needs to be measured using a risk-benefit approach

Special patient groups

Good patient communication is essential for the management of COPD, both for proper use of medications (especially inhalers) and monitoring symptoms. Special intervention needs to be provided for appropriate patients, e.g. interpreters A subset of patients with COPD are candidates for lung volume reduction surgery or lung transplantation. If these surgical interventions are contemplated, a referral to a specialist with experience in these issues is appropriate

Patient satisfaction/lifestyle priorities

Coughing may be socially embarrassing, particularly for women with stress incontinence Dyspnea severe enough to limit activities can lead to depression related to loss of previous lifestyle Many patients may be frightened to give up the smoking 'crutch' they have relied on for most of their lives and may adamantly reject the idea of smoking cessation; be aware of patients not telling the truth just to make the health care provider happy Pills or liquid therapy may be preferred over use of inhalers because inhalers require precise patient technique for proper use; with increasing age, patients may not be able to use inhalers properly; pills are also less conspicuous Spacer devices may augment the patient's ability to use metered dose inhalers optimally
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Patient and caregiver issues

Impact on career, dependants, family, friends
Dyspnea may significantly limit patients' ability to perform activities of daily living, and may restrict their ability to participate in leisure-time activities; families need to know how to work around the patients' limitations so the patient can still be involved in family activities Many patients learning to live with chronic illness may become depressed and withdrawn; family members need to know about this and report serious depression to the primary care provider if the patient is unable to do so Some family members may resist smoking cessation when around the person with COPD; this may cause friction in the family, particularly if they share the same dwelling The person with COPD may have increased absences from work, or may need to consider retirement or change of duties as the disease progresses. There may be a significant impact on financial situation (e.g. missed work, increased cost for medications, caretakers) Family and social supports are essential in patients with severe disease

Questions patients ask

Is it too late for me to quit smoking? The damage has already been done. Actually, within 24h of quitting, there are positive effects in the body and eliminating exposure to cigarette smoke significantly limits further lung damage. Patients may be incredibly threatened by the suggestion that they stop smoking, especially if they are using smoking to 'self-medicate' anxiety and/or depression I switched to cigars (or a pipe and tobacco or smokeless tobacco) so I don't have any 'real' exposure to tobacco smoke. Not only do cigars and pipes produce inhaled irritants, but they are also associated with mouth cancer. Smokeless tobacco is also associated with an increased risk for mouth cancer But I have friends who smoked for years, and they're fine! A number of factors interact to cause COPD; cigarette smoke is a very important factor, but so are other things such as environmental exposure, genetics, and a history of lung infections Does this mean I'm going to get lung cancer? Some people who have COPD get lung cancer, and some don't. It's difficult to predict. Increasing dyspnea and hemoptysis require further investigation I just clear my throat in the morning, I don't have a cough. At the time of diagnosis, many people will try to minimize their symptoms because they cannot face the thought

that they now have a chronic, progressive illness, particularly one related to a habit like smoking

Health-seeking behavior
Have you ever thought about quitting smoking? Have you tried to quit before? Explain that few people are successful quitting the first time they try, particularly if they were not in a structured program or did not have pharmacologic support. Encourage them to try again, this time with your help Is there someone you could quit with? Quitting at the same time as a spouse or other family member is important because the people can support each other. It is very difficult for a patient to stop smoking when other people sharing the dwelling are still smoking Do you get a flu shot every year? Influenza vaccines help reduce complications of COPD Have you had a pneumococcal vaccine? The pneumococcal vaccine should be administered every five years for patients with COPD or other chronic lung diseases
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Follow-up
Plan for review
According to GOLD practice guidelines, ongoing monitoring and assessment of COPD patients should include evaluation of: Continued exposure to inhaled irritants, particularly cigarette smoke (but may also include occupational and environmental exposures, depending on the individual) Disease progression and development of complications: best tracked by office spirometry, physical examination, and other tests as indicated by individual assessment Effectiveness of pharmacotherapy and other medical treatment(s) with regard to symptom relief Exacerbation history through questioning patient about self-treated episodes and those treated by other health care providers (walk-in center or emergency room) Comorbidities, such as bronchial carcinoma, pulmonary tuberculosis, sleep apnea, and heart failure

Information for patient or caregiver

Teach the patient the following factors regarding exposure control (individualize based on the type of exposure): Avoid vigorous outdoor activity during high pollution episodes Ensure adequate room ventilation if solid fuels are used for cooking or heating Monitor public announcements and stay indoors if air quality is poor Do not adjust medications (including oxygen) without specific instructions from a health care provider Do not spend a lot of money on consumer air cleaners; they have not been shown to have significant health benefits Patients with chronic hypoxia should not travel to locations at altitudes >4000ft

General education topics:

How to reduce risk factors Smoking cessation Improve health management skills How to use inhaler properly (if prescribed) Teach how to cope with chronic illness Ways to minimize dyspnea What to do if symptoms significantly increase (acute exacerbation) Advance directives and end-of-life decisions
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Ask for advice

Question 1
My patient asked me, 'why should I stop smoking? My uncle is 90 years old and has smoked since he was a teenager'. How important is smoking cessation in COPD?

Answer 1
Only about 15-20% of smokers develop frank COPD. Smokers often modify their activities to compensate for loss of lung function well past the onset of airway obstruction and deny symptoms. Thus, pulmonary function testing is crucial for diagnosis and, if obstruction is documented, can be used to aid in smoking cessation. Even in patients without frank COPD, loss of lung function is accelerated. Cigarette smoking remains a very significant risk factor for atherosclerosis and the primary risk factor for lung cancer, underscoring the need for smoking cessation.

Question 2
What can my patient do to get his lung function back?

Answer 2
Short of the small minority of patients who are candidates for surgical intervention, very little can be done to regain lost lung function. Smoking cessation and implementation of bronchodilators lead to a rapid, but modest improvement in the FEV1. Smoking cessation does lead to a decrease in the rate of decline of FEV 1. Pulmonary rehabilitation can optimize activities of daily living, but does not improve pulmonary function.

Question 3
My patient can tell when she needs oxygen by how she feels. Must she use it even when she's not short of breath?

Answer 3
Yes, the Nocturnal Oxygen Therapy Trial demonstrated that outcomes are better when oxygen, when indicated, is used for as close to 24h/day as possible.

Question 4
What role does pulmonary rehabilitation have for my patient? He already exercises.

Answer 4
Pulmonary rehabilitation provides important education regarding the pathophysiology of COPD, its treatment, and living with COPD. Careful monitoring establishes a safe level of

exercise, which avoids complications of exercise but also encourages the patient to exercise beyond levels limited only by dyspnea.

Question 5
Will my patient need oxygen during air flight?

Answer 5
Aircrafts are typically pressurized to between 5000 and 7000ft (1500-2100m). However, supplementing oxygen to account for pressurization to 8000ft (2438m) is recommended. As a rule of thumb, a decrement of 2-3L of O2 flow via nasal cannulae will suffice to replace the inspired O2 lost at 8000ft.
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Consider consult
Referral to a respiratory physician may be required in patients with moderate (FEV1 <50% of predicted) to severe (FEV1 <30% of predicted) COPD, hypoxia, or persistent heavy sputum production Consider referral if existing cardiac disease complicates COPD, or if cor pulmonale results from COPD

Outcomes
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Prognosis
Chronic obstructive pulmonary disease (COPD) has a variable natural history It is generally a progressive disease, especially if exposure to inhaled irritants continues (i.e. the patient does not stop smoking) Stopping exposure can slow or even halt the progression of the disease (excluding typical changes in lung function associated with aging) Use of home oxygen in patients in chronic respiratory failure increases survival COPD is the fourth or fifth leading cause of death in the US in those over age 45 (depending on how the numbers are calculated, using chronic bronchitis and emphysema data)

Clinical pearls
Recent trials suggest that inhaled corticosteroids should be reserved for patients with COPD with an FEV1of 1L or less Home-assisted positive-pressure ventilation has been suggested as an intervention to aid with CO2retention and to rest fatigued respiratory muscles. However, the efficacy of home-assisted positive-pressure ventilation is controversial and major complications are possible. If contemplated, home-assisted positive-pressure ventilation should only be initiated by a center with experience

Progression of disease

Therapeutic failure
If initial bronchodilator therapy fails with a beta-2 agonist, add an anticholinergic bronchodilator If combination bronchodilator therapy fails, and the patient has a proven response to glucocorticosteroids, try adding inhaled corticosteroids If these three approaches have not improved symptoms, consider referral to pulmonary specialist for additional diagnostics and therapy recommendations to manage stable COPD Exacerbations will occur as a result of infection and air pollution exposure (yet the cause of one-third of exacerbations is unknown); this is not considered therapeutic failure

Recurrence
COPD is a chronic, persistent illness; no 'recurrence' is possible

Deterioration
For severe COPD, home oxygen may be used to reduce symptoms and try to relieve hypoxemia.

Terminal illness
The primary health care provider should open a discussion with the patient with moderate or severe COPD about his or her wishes for therapeutic interventions, particularly mechanical ventilation in case of respiratory failure The primary provider may need guidance from a pulmonary specialist regarding the potential for weaning a patient with severe disease from a ventilator if the patient expresses a wish 'not to be hooked up to machines for the rest of my life' It is important for the primary care provider to initiate this discussion and be clear about the patient's wishes. Remind the patient to execute a living will and durable power of attorney for health care to legally reflect his or her wishes In end stages of COPD, hospice care may be considered; while no randomized controlled studies have been found, there are a number of anecdotal reports about the use of nebulized opioids to relieve dyspnea in the terminally ill patient
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Clinical complications
Cor pulmonale with heart failure Pneumothorax Respiratory failure Arrhythmias, including atrial fibrillation Infection Secondary polycythemia
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Consider consult
If the initial treatment approaches have not improved symptoms, consider referral to pulmonary specialist for additional diagnostics and therapy recommendations to manage stable COPD.

Prevention
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Primary prevention
Modifiable risk factors Tobacco
Smoking cessation is critical to future prevention of chronic obstructive pulmonary disease (COPD) Exposure to second-hand smoke can also place people at risk for developing COPD

Environment
Important to limit exposure to inhaled particles and gases, whether in the occupational setting or at home (fuels used for heating and cooking, air pollution).

Immunization
Patients with COPD should have the following vaccinations if no contraindications exist: Influenza yearly Pneumococcal vaccine every 5 years

Other
People with family members with symptoms of significant COPD before age 40 (particularly in the absence of cigarette smoking) may wish to consider testing for alpha-1-antitrypsin deficiency.
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Secondary prevention [EBM]

To prevent recurrence of exacerbations, patients with COPD must: stop smoking; limit environmental exposure to vapors, irritants, and fumes; get influenza and pneumococcal vaccines; and limit exposure to people with respiratory illnesses.

Evidence
A systematic review of RCTs of live or inactivated influenza vaccines vs placebo found that inactivated vaccines reduce exacerbations in patients with COPD. The reviewers note, however, that the number of RCTs is limited and that not all of the studies were conducted solely in COPD patients - some included elderly, high-risk people with or without chronic lung diseases. The studies are too small to detect any effect on mortality [[28]]Level A A systematic review of RCTs of injectable pneumococcal vaccines found that the effects in terms of reducing the risk of acute exacerbations did not reach statistical significance [[29]]Level A

Screening
Patients aged 45 and older who smoke (quit during the previous year) or have significant environmental exposure to inhaled particles and gases should have office spirometry performed as a screening measure.