Clinical Presentations and Complications of Emphysema
At one extreme of emphysema, patients exhibit pronounced chronic bronchitis leading to hypoxia and cyanosis, while at the other extreme, obese patients with chronic heart failure seek medical help. Emphysema-related conditions include compensatory emphysema, obstructive overinflation, and bullous emphysema, each with specific characteristics and risks such as secondary pulmonary hypertension.
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Presentation Transcript
- At the other extreme of the clinical presentation in emphysema is a patient who also has pronounced chronic bronchitis - Dyspnea usually is less prominent, with diminished respiratory drive, so the patient retains carbon dioxide, becomes hypoxic, and often is cyanotic. 2
- For reasons not entirely clear, such patients tend to be obese-hence the designation "blue bloaters. - They seek medical help after the onset of CHF (cor pulmonale) and associated edema 3
Note : - In all cases, secondary pulmonary hypertension develops arising from both hypoxia-induced pulmonary vascular spasm and loss of pulmonary capillaries - Death from emphysema is related to either pulmonary failure, with respiratory acidosis, hypoxia, and coma, or right-sided heart failure (cor pulmonale). 4
Conditions Related to Emphysema - Several conditions resemble emphysema only superficially but nevertheless are (inappropriately) referred to as such: I. Compensatory emphysema - Is a term used to designate the compensatory dilation of alveoli in response to loss of lung substance, such as occurs in residual lung parenchyma after surgical removal of a diseased lung or lobe. 5
II. Obstructive overinflation - The condition in which the lung expands because air is trapped within it. - A common cause is subtotal obstruction by a tumor or foreign object. - Obstructive overinflation can be a life- threatening emergency if the affected portion extends sufficiently to compress the remaining normal lung. 6
III. Bullous emphysema - Refers to any form of emphysema that produces large subpleural blebs or bullae (spaces greater than 1 cm in diameter in the distended state). 7
and these blebs represent localized accentuations of any form of emphysema; most often the blebs are subpleural, and on occasion they may rupture, leading to pneumothorax 8
IV. Mediastinal (interstitial) emphysema - Is the condition resulting when air enters the connective tissue stroma of the lung, mediastinum, and subcutaneous tissue. 9
Causes 1.Spontaneously with a sudden increase in intra-alveolar pressure (vomiting or violent coughing) in children with whooping cough 2. Occurs in patients on respirators who have partial bronchiolar obstruction 3. In persons who suffer a perforating injury (e.g., a fractured 10
- When the interstitial air enters the subcutaneous tissue, the patient may blow up like a balloon, with marked swelling of the head and neck and crackling crepitation all over the chest. - In most instances, the air is resorbed spontaneously after the site of entry is sealed. 11
2. Chronic Bronchitis - Is common among cigarette smokers and urban dwellers - The diagnosis of chronic bronchitis is made on clinical grounds: - It is defined by the presence of a persistent productive cough for at least 3 consecutive months in at least 2 consecutive years. 12
Note -In early stages of the disease, the productive cough raises mucoid sputum, but airflow is not obstructed. - Some patients with chronic bronchitis may have hyper- responsive airways with intermittent bronchospasm and 13
- A subset of bronchitic patients, especially heavy smokers, develop chronic outflow obstruction, usually with associated emphysema 14
PATHOGENESIS - The distinctive feature of chronic bronchitis is hypersecretion of mucus, beginning in the large airways. - Although the single most important cause is cigarette smoking, other air pollutants, such as sulfur dioxide and nitrogen dioxide, may contribute. 15
- These environmental irritants a. Induce hypertrophy of mucous glands in the trachea and main bronchi, b. Marked increase in mucin-secreting goblet cells in the surface epithelium of smaller bronchi and bronchioles. C. Infiltration of CD8+ lymphocytes, neutrophils but no eosinophils 16
- It is postulated that many of the respiratory epithelial effects of environmental irritants (mucus hypersecretion) are mediated by local release of T- cell cytokines such as IL-13. - The transcription of the mucin gene MUC5AC in bronchial epithelium is a consequence of exposure to tobacco smoke. 17
Note - The defining feature of chronic bronchitis (mucus hypersecretion) is primarily a reflection of large bronchial involvement - The morphologic basis of airflow obstruction in chronic bronchitis is more peripheral and results from: 18
(1) Small airway disease (chronic bronchiolitis) is induced by: a. Goblet cell metaplasia with mucous plugging of the bronchiolar lumen, b. Inflammation, c. Bronchiolar wall fibrosis, (2) Coexistent emphysema 19
. Note - Small airway disease ( chronic bronchiolitis) is an important component of early and relatively mild airflow obstruction, - Significant airflow obstruction is almost always caused by emphysema - chronic bronchitis with 20
MORPHOLOGY Gross: - Hyperemia and swelling of the mucosal lining of the large airways. - The mucosa of bronchi is covered by a layer of mucinous or mucopurulent secretions 21
On histologic examination - Enlargement of the mucus-secreting glands in trachea and large bronchi. - The magnitude of the increase in size is assessed by the ratio of the thickness of the submucosal gland layer to that of the bronchial wall (the Reid index-normally 0.4). 22
- Inflammatory cells, largely mononuclear but sometimes admixed with neutrophils, are frequently present in variable density in the bronchial mucosa . 23
. - It is the submucosal fibrosis that leads to luminal narrowing and airway obstruction. - Changes of emphysema often co- exist 24
Clinical Features - In patients with chronic bronchitis, a prominent cough and the production of sputum may persist indefinitely without ventilatory dysfunction - Some patients develop significant COPD with outflow obstruction. 25
- This clinical syndrome is accompanied by hypercapnia, hypoxemia, and (in severe cases) cyanosis (hence the term "blue bloaters"). - With progression, chronic bronchitis is complicated by pulmonary hypertension and cardiac failure . - Recurrent infections and respiratory failure are constant threats. 26
