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acute interstitial pneumonia; Hamman-Rich syndrome; idiopathic alveolar fibrosis; rapidly progressive pulmonary fibrosis (IAF)
diagnosis of exclusion
Etiology:
- patients lack risk factors for ARDS
Pathology:
- diffuse alveolar damage
Clinical manifestations:
- acute onset, days to weeks
- fulminant respiratory failure (ARDS)*
- nail clubbing
- early death
* clinically, pathologically & radiographically indistinguishable from ARDS [1]
Radiology:
- chest X-ray
- chest CT
- diffuse ground glass appearance with consolidation
Complications:
- short-term mortality is 50%
Management:
- glucocorticoids are used, although evidence of benefit is anecdotal
- intensive care unit
- mechanical ventilation
- low tidal volume (6 mL/kg of ideal body weight)
- patients who recover may relapse or develop chronic interstitial lung disease
General
interstitial pneumonia
pulmonary fibrosis
References
- Medical Knowledge Self Assessment Program (MKSAP) 14, 16, 17, 18, 19.
American College of Physicians, Philadelphia 2006, 2012, 2015, 2018, 2022.