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Chronic Obstructive Pulmonary Disease and Emphysema
Alan Cropp, MD, FCCP
Michael Apostolis, MD
image BASICS
  • Chronic obstructive pulmonary disease (COPD) usually describes a mixture of chronic bronchitis and emphysema. It is characterized by airflow limitation that is not fully reversible, inflammation, and is progressive (1,2).
  • Chronic bronchitis is defined clinically by increased mucus production and recurrent cough present on most days for at least 3 months per year during at least two consecutive years without another cause.
  • Emphysema, the destruction of interalveolar septa, occurs in the distal or terminal airways and involves both airways and lung parenchyma.
Affects more than 5% of adults in the United States and is the 12th leading cause of morbidity (1)
  • Third leading cause of death in the United States (1)
  • Projected to be the third leading cause of death globally by 2020 (2)
Cigarette and/or cannabis smoking, air pollution (including indoor), antiprotease deficiency (&agr;1-antitrypsin), occupational exposure (firefighters), infection (viral), occupational pollutants (cadmium, silica)
  • Impaired gas (carbon dioxide and oxygen) exchange
  • Chronic bronchitis: airway obstruction (3)
  • Emphysema: destruction of lung parenchyma
  • Chronic bronchitis is not a genetic disorder.
  • Antiprotease deficiency (due to &agr;1-antitrypsin deficiency) is an inherited, rare disorder due to two autosomal codominant alleles.
  • Smoking: passive smoking, especially adults whose parents smoked; cannabis use (one joint is equivalent to 2.5 to 5 cigarettes) (1,2,3)
  • Severe pneumonia early in life including viral (4)
  • Aging
  • Lower level of education and poverty (4)
  • Airway hyperactivity
  • Indoor pollution (open fire for cooking or heating) (4)
  • Occupational organic or inorganic dusts (4)
  • Avoidance of smoking is the most important preventive measure.
  • Early detection through pulmonary function tests (PFTs) in high-risk patients may be useful in preserving remaining lung function.
  • Pulmonary: lung cancer, chronic respiratory failure, acute bronchitis, sleep apnea, pulmonary hypertension, asthma
  • Cardiac: coronary artery disease, arrhythmia
  • Ear, nose, and throat (ENT): chronic sinusitis, laryngeal carcinoma
  • Miscellaneous: malnutrition, osteoporosis, muscle dysfunction, depression
  • Rarely diagnostic for COPD (2)
  • Chronic bronchitis: cyanosis, wheezing, weight gain, diminished breath sounds, distant heart sounds
  • Emphysema: barrel chest, minimal wheezing, accessory muscle use, pursed lip breathing, cyanosis slight or absent, breath sounds diminished
  • Asthma (including occupational)
  • Bronchiectasis
  • Lung cancer
  • Acute bronchitis
  • Normal aging of lungs
  • Chronic pulmonary embolism
  • Sleep apnea
  • Primary alveolar hypoventilation
  • Chronic sinusitis
  • Reactive airways dysfunction syndrome (RADS)
  • Congestive heart failure (CHF)
  • Bronchiolitis obliterans
  • Gastroesophageal reflux disease
  • Cystic fibrosis
Spirometry (the most reliable/objective measurement of airflow obstruction) (1,2,3)[A]
Initial Tests (lab, imaging)
  • Spirometry (1,2,3) (see “Diagnostic Procedures”)
  • Chronic bronchitis
    • Arterial blood gases (ABGs) may show hypercapnia and hypoxia.
    • Hemoglobin may be increased.
  • Emphysema
    • Normal serum hemoglobin or polycythemia
    • Normal PaCO2 on ABGs unless forced expiratory volume in 1 second (FEV1) <1 L, in which case it can be elevated
    • Mild hypoxia
  • Chronic bronchitis CXR: increased bronchovascular markings and cardiomegaly
  • Emphysema CXR: small heart, hyperinflation, flat diaphragms, and possibly bullous changes
Follow-Up Tests & Special Considerations
  • Check continuous overnight oximetry in selected patients.
  • &agr;1-Antitrypsin screening for those with COPD age <45 years, have a blood relative with this disease, or spirometry out of proportion to tobacco use
  • Chest CT may show diffuse bullous changes or upper lobe predominance. Also, small lung nodules may be identified (5).
Diagnostic Procedures/Other
  • PFTs (1,2)[A]
    • Not indicated during acute exacerbation (3)
    • Decreased FEV1 and resulting reduction in FEV1: FVC (forced vital capacity) ratio
    • Poor or absent reversibility to bronchodilator
    • Normal or reduced FVC
    • Normal or increased total lung capacity
    • Increased residual volume and functional residual capacity
    • Diffusing capacity is normal or reduced.
  • Staging: Global Initiative for Obstructive Lung Disease (GOLD) criteria (3)
    • Mild COPD: FEV1 ≥80% predicted
    • Moderate COPD: FEV1 50-80% predicted
    • Severe COPD: FEV1 30-50% predicted
    • Very severe COPD: FEV1 <30% predicted or FEV1 <50% predicted plus chronic respiratory failure
Test Interpretation
  • Chronic bronchitis: bronchial mucous gland enlargement, increased number of secretory cells in surface epithelium, thickened small airways from edema and inflammation, smooth muscle hyperplasia, mucus plugging, bacterial colonization of airways
  • Emphysema: entire lung affected, bronchi usually clear of secretions, anthracotic pigment, alveoli enlarged with loss of septa, cartilage atrophy, bullae
  • Smoking cessation: This is the most important intervention to decrease risk (1,2)[A].
  • Aggressive treatment of infections (3)[B]
  • Treat any reversible bronchospasm.
  • Home oxygen: may improve survival; should be initiated partial pressure of arterial oxygen (PaO2) ≤55 mm Hg or pulse oximetry trends ≤88% (1)[A]
  • Influenza and pneumococcal immunizations (2)[B]
  • Tiotropium (Spiriva) may slow disease progression (6)[B].
Medications help reduce symptoms and exacerbations (1).
First Line
  • All patients should have a short-acting &bgr;-agonist (albuterol) to use as a rescue drug (1).
  • Anticholinergics (2)[A]
    • Ipratropium (Atrovent), tiotropium (Spiriva), aclidinium bromide (Tudorza) AND/OR
  • Long-acting &bgr;-agonists
    • Salmeterol (Serevent), formoterol (Foradil), one inhalation q12h; or arformoterol (Brovana), formoterol (Perforomist), nebulized q12h (1,2)[A]
Second Line
  • Trial of inhaled corticosteroids for moderate or severe disease (2)[A]
    • May initiate earlier if suggestion of asthmatic component to disease
    • Systemic corticosteroids: Prednisone (Deltasone) can be given orally 7.5 to 15 mg/day in selected patients.
    • P.201

    • Long-term monotherapy with steroids (oral or inhaled) is not recommended (2,3)[A].
    • Inhaled corticosteroids are associated with an increased risk of pneumonia (3).
  • Continuous home oxygen: may improve survival; should be initiated for severe resting hypoxemia (PaO2≤55 mm Hg or oxygen saturation ≤88%) or PaO2 56 to 59 mm Hg with evidence of hypoxia (i.e., polycythemia or pulmonary hypertension) or pulse oximetry trends ≤88% (1)[A]
  • Theophylline if other long-term treatment is unavailable or unaffordable: 400 mg/day; increase by 100 to 200 mg in 1 to 2 weeks, if necessary (4)[B]
    • Reduce dosage in patients with impaired renal or liver function, age >55 years, or CHF
    • Monitor serum level. Therapeutic range is 8 to 13 &mgr;g/mL.
    • Low-dose theophylline may help inflammatory component (4)[B].
  • Combination of inhaled corticosteroid, long-acting &bgr;-agonist, and anticholinergic indicated for severe disease. Several combination medications are now available (2)[A].
  • Mucolytic agents may improve secretions but do not improve outcomes.
  • Phosphodiesterase-4 inhibitor (PDE4) inhibitor (roflumilast) in severe chronic bronchitis may reduce exacerbations (2)[B].
  • &agr;1-Antitrypsin, if deficient: 60 mg/kg/week to maintain level >80 mg/dL
  • Precautions
    • Sympathomimetics: excessive use may be dangerous. May need to reduce dosage or use levalbuterol (Xopenex) in patients with cardiovascular disease, hypertension (HTN), hyperthyroidism, diabetes, or seizures. Anticholinergics: narrow-angle glaucoma, benign prostatic hyperplasia, bladder neck obstruction
    • Corticosteroids: weight gain, diabetes, adrenal suppression, osteoporosis, infection (pneumonia)
  • Sympathomimetics may be aerosolized.
  • Anticholinergics: Ipratropium (Atrovent) may be aerosolized or combined with albuterol (Combivent Respimat).
Severe exacerbation, frequent hospitalizations, age <40 years, rapid progression, weight loss, severe disease, or surgical evaluation
  • Adequate hydration and pulmonary hygiene
  • Consider postural drainage, flutter valve, or other devices to assist mucus clearance.
  • Pulmonary rehabilitation (1,2,3)[A]
  • Intermittent, noninvasive ventilation may help in severe chronic respiratory failure.
  • Short course of antibiotics (5 to 10 days) for acute exacerbations (2)[B]
  • Immunizations
  • Supplemental oxygen if indicated
  • Lung reduction surgery (selected cases)
  • Lung transplantation (selected cases)
Admission Criteria/Initial Stabilization
  • Outpatient treatment is usually adequate.
  • Exacerbation with acute decompensation (hypoxemia, hypercarbia)
  • Serious comorbidities (i.e., decompensated CHF)
  • Systemic steroids reduce recovery time and improve hypoxia (2,7)[A]
  • Supplemental oxygen and short-acting bronchodilators should be given (3).
  • www.agingwithdignity.org, www.putitinwriting.org
  • Progressive nature of disease and severity of treatment methods (ventilation, etc.) make revisiting patient preferences beneficial.
  • Acute respiratory failure may require ICU and invasive or noninvasive ventilation (NIV) (2)[A].
  • Systemic steroids (prednisone 40 mg/day for 5 days or equivalent) have been shown to reduce recovery time and improve hypoxia (7)[A].
Teach proper inhaler use.
Discharge Criteria
  • Ability to ambulate (3)
  • Hypoxia can be treated with home oxygen (may only be temporary) (2)[A].
  • Inhaled short-acting &bgr;-agonist therapy no more frequently than q4h (3)
  • Ability to eat and sleep without interruption caused by dyspnea (3)
  • May taper or stop oral steroids as outpatient
  • If pneumonia caused exacerbation, need to follow CXR or chest CT until clear or stable
  • Pulmonary rehabilitation for exertional dyspnea (1)[A]
Patient Monitoring
  • Severe or unstable patients should be seen monthly. When stable, see every 6 months.
  • Check theophylline level with dose adjustment, then check every 6 to 12 months.
  • With use of home oxygen, check ABGs yearly or with change in condition. Frequently monitor saturation (pulse oximetry). Some patients only desaturate at night, thus only need nocturnal oxygen.
  • Yearly spirometry
  • Travel at high altitude with supplemental oxygen if necessary.
  • Baseline chest CT for patients aged 55 to 74 years with a 30 pack/year smoking history to look for lung nodules (5)[B]
  • Discuss advance directive and health care proxy.
A high-protein low-carbohydrate diet may benefit those with hypercarbia.
American Lung Association: www.lung.org/lung-disease/copd/
  • Patient's age and postbronchodilator FEV1 are the most important predictors of prognosis.
  • Supplemental O2, when indicated, is shown to increase survival (may only need at night) (1)[A].
  • Smoking cessation improves prognosis—consider E-cigarettes (2).
  • Malnutrition, cor pulmonale, hypercapnia, and pulse >100 indicate a poor prognosis.
1. Qaseem A, Wilt TJ, Weinberger SE, et al. Diagnosis and management of stable chronic obstructive pulmonary disease: a clinical practice guideline update from the American College of Physicians, American College of Chest Physicians, American Thoracic Society, and European Respiratory Society. Ann Intern Med. 2011;155(3):179-191.
2. Vestbo J, Hurd SS, Agustí AG, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med. 2013;187(4):347-365.
3. Global Initiative for Chronic Obstructive Lung Disease. Global strategy for the diagnosis, management, and prevention of COPD. Revised 2013. http://www.goldcopd.org/guidelines-global-strategy-for-diagnosis-management.html
4. Lamprecht B, McBurnie MA, Vollmer WM, et al. COPD in never smokers: results from the population-based burden of obstructive lung disease study. Chest. 2011;139(4):752-763.
5. Detterbeck FC, Mazzone PJ, Naidich DP, et al. Screening for lung cancer: diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2013;143(5)(Suppl):e78S-e92S.
6. Csikesz NG, Gartman EJ. New developments in the assessment of COPD: early diagnosis is key. Int J Chron Obstruct Pulmon Dis. 2014;9:277-286.
7. Leuppi JD, Schuetz P, Bingisser R, et al. Short-term vs conventional glucocorticoid therapy in acute exacerbations of chronic obstructive pulmonary disease: the REDUCE randomized clinical trial. JAMA. 2013;309(21):2223-2231.
See Also
Bronchitis, Acute
  • J44.9 Chronic obstructive pulmonary disease, unspecified
  • J43.9 Emphysema, unspecified
  • J42 Unspecified chronic bronchitis
Clinical Pearls
  • Consider screening PFTs on any high-risk patient.
  • Overnight oximetry if daytime SaO2 is borderline.
  • Influenza/pneumococcal vaccines should be current.
  • Advance directive before patient is seriously ill.
  • Consider chest CT for patients age 55 to 74 years with a 30 pack/year smoking history for lung cancer screening.