The most common cause has historically been, and unfortunately continues to be, smoking. It takes many years of smoking to develop COPD and as such typically patients are older adults. There are however a number of other less common risk factors/aetiologies, each with their own demographics. They include:
- cigarette smoking
- industrial exposure (e.g. mining)
- cystic fibrosis
- alpha-1 antitrypsin deficiency
- intravenous drug use
- immune deficiency syndromes
- vasculitides and connective tissue disorders
Symptoms of COPD include dyspnoea on exertion, wheezing, productive cough, pursed-lip breathing, and use of accessory muscles. Patients with chronic bronchitis are classically "blue bloaters," while those with emphysema are known as "pink puffers". In advanced cases, muscle wasting, asterixis, and peripheral oedema may be seen.
In contrast to asthma, the histologic changes of COPD are irreversible and gradually progress over time. In chronic bronchitis, there is diffuse hyperplasia of mucous glands with associated hypersecretion and bronchial wall inflammation.
Emphysema involves the destruction of alveolar septa and pulmonary capillaries, leading to decreased elastic recoil and resultant air trapping. The morphological subtypes of emphysema include:
- centrilobular (centriacinar): associated with smoking and spreads peripherally from bronchioles
- panacinar: homozygous AAT1 deficiency and uniformly destroys alveoli
- paraseptal (distal acinar): involves the distal airways
Pulmonary function testing (PFT) reveals airflow obstruction, as evidenced by a decreased forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio. Administration of bronchodilators has no effect, unlike the reversible obstruction seen in asthma.
The global initiative for chronic obstructive lung disease (GOLD) staging system is a commonly used severity staging system based on airflow limitation. According to this, there are 4 key stages:
- stage I: mild, FEV1 > 80% of normal
- stage II: moderate, FEV1 = 50-79% of normal
- stage III: severe, FEV1 = 30-49% of normal
- stage IV: very severe, FEV1 <30% of normal or <50% of normal with presence of chronic respiratory failure present
The FEV1:FVC ratio should be <0.70 for all stages.
The GOLD staging system may be insensitive in early stages 12.
Several distinct clinical phenotypes have been described 4,6,8:
- emphysema predominant
- airways predominant
- small airways predominant
- large airways predominant
Findings of chronic bronchitis on chest radiography are non-specific and include increased bronchovascular markings and cardiomegaly. Emphysema manifests as lung hyperinflation with flattened hemidiaphragms, a small heart, and possible bullous changes. On the lateral radiograph, a "barrel chest" with widened anterior-posterior diameter may be visualized. The "saber-sheath trachea" sign refers to marked coronal narrowing of the intrathoracic trachea (frontal view) with concomitant sagittal widening (lateral view).
Findings of COPD may be seen in a variety of CT chest studies, e.g. contrast-enhanced CT, CT pulmonary angiography, staging CT chest, HRCT chest, etc.
In chronic bronchitis, bronchial wall thickening may be seen in addition to enlarged vessels. Repeated inflammation can lead to scarring with bronchovascular irregularity and fibrosis.
Emphysema is diagnosed by alveolar septal destruction and airspace enlargement, which may occur in a variety of distributions. Centrilobular emphysema is predominantly seen in the upper lobes with panlobular emphysema predominating in the lower lobes. Paraseptal emphysema tends to occur near lung fissures and pleura. Formation of giant bullae may lead to compression of mediastinal structures, while rupture of pleural blebs may produce spontaneous pneumothorax/pneumomediastinum.
Treatment and prognosis
Barring whole-lung transplantation, there is currently no cure for COPD, but it is highly preventable and treatable.
Risk factor reduction via smoking cessation, occupational health, and air pollution reduction should be instituted. Patients should also have all available vaccinations.
Pharacological management is generally first-line. It involves the use of bronchodilators, corticosteroids, and other medications (e.g. methylxanthines, leukotriene receptor antagonists, phosphodiesterase type-4 inhibitors, omalizumab), as well as supplemental oxygen and pulmonary rehabilitation. Long-acting β2-agonist (LABA) and long-acting muscarinic antagonist (LAMA) combination therapies are currently considered the most effective strategy 15.
Acute exacerbations are treated with high-dose corticosteroids, short-acting bronchodilators, supplemental oxygen, and antibiotics if indicated.
Surgical therapy is usually reserved for COPD refractory to pharmacological management. In addition to the aforementioned whole-lung lung transplant, other surgical procedures include:
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