Abstract

Interstitial lung fibrosis/cryptogenic fibrosing alveolitis is a chronic progressive lung disorder characterized by interstitial inflammation and fibrosis of the lung parenchyma. The etiology and pathogenesis are unknown. The role of occult environmental agents in development of IPF has remained unclear. IPF carries a 50% 5 yr survival rate. The diagnosis of IPF is difficult, being often a diagnosis of exclusion. There are major and minor diagnostic criteria. Clinical symptoms, X-ray, CT-scan, BAL, and often even open lung biopsy are not specific for the diagnosis. The prognosis of IPF is poor and the survival rate is low. The effect of treatment is often insufficient and assessment of its efficacy is complicated.