Abstract
The most reasonable classification for diffuse interstitial pneumonia/fibrosis is described according to the clinical course, histopathological findings and responses to steroids. Idiopathic pulmonary fibrosis (IPF) represents the most prevalent, most chronic form of the disease with the worst prognosis. The histopathological findings of IPF are usual interstitial pneumonia (UIP) which microscopically is recognized as patchy lesions. In UIP diffuse alveolar inflammation does not change to diffuse alveolar fibrosis, rather the disease progresses via an increase in the number of localized fibrosis lesions.
Steroids are ineffective in the treatment of IPF. Lung transplantation is the only effective treatment. The question of when such transplantation should be undertaken constitutes the subject of further study.
