Progressive inflammation, fibrosis, and destruction of lung (end-stage lung) of unknown cause. Synonyms: usual interstitial pneumonia (UIP, pathology term), cryptogenic fibrosing alveolitis (British term). Hamman-Rich syndrome is an acute, rapidly fatal form of IPF, and IPF has been termed "chronic Hamman-Rich syndrome." Prognosis: mean survival 4 years (range 0.4 to 20 years). Lung biopsy is necessary for diagnosis. Treatment: steroids, cytotoxic agents.
Clinical Findings:
-clubbing
-nonproductive cough
-dyspnea
-weight loss
Radiographic Features:
Distribution:
-primarily lower lung zones and peripheral subpleural involvement.
HRCT pattern:
-early: ground glass attenuation
-later: reticular pattern in lower lobes
-end-stage: honeycombing
-traction bronchiectasis indicates fibrosis