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bullous lung disease
Etiology:
1) small apicial bullae may be present in the lung apex of normal individuals
2) congenital
a) Marfan's syndrome
b) Ehlers-Danlos syndrome
3) acquired
a) burnt out sarcoidosis
b) cadmium exposure
4) panlobular emphysema may appear like bullae
5) bullae may occur spontaneously or, more frequently, in the setting of obstructive lung disease (pulmonary emphysema) [2]
Pathology:
- lack of air communication with bronchi may cause air trapping
Clinical manifestations:
- chronic dyspnea without cough
Special laboratory:
- pulmonary function testing
- obstructive lung disease
- no response to bronchodilator [2]
Radiology:
- chest X-ray
- sharply demarcated air-containing spaces within the lung, surrounded by a smooth wall (bullae)
- hyperlucent spaces, & hairline shadows often form their borders & distinguish them from surrounding lung
- bullae are not always apparent on plain chest radiographs
- absence of lung markings in a lung zone suggests a bulla
- hyperinflation, flattened diaphragm, & an increased retrosternal air space suggests bullous lung disease in the setting of pulmonary emphysema [2]
Complications:
1) pneumothorax
2) COPD
3) lung abscess
4) hemorrhage into bulla
5) compression of adjacent normal lung
Differential diagnosis:
- bronchiectasis
- pulmonary emphysema
Management:
- surgery may improve lung function by 5-10% in 10-15% of patients
Related
bulla; bleb
Ehlers-Danlos syndrome; cutis elastica (EDS)
emphysema
Marfan syndrome
sarcoidosis
Specific
congenital cystic adenomatoid malformation; congenital pulmonary airway malformation
giant bullous emphysema; Vanishing lung syndrome
General
lung disease
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 739-40
- Medical Knowledge Self Assessment Program (MKSAP) 20
American College of Physicians, Philadelphia 2025