pulmonary disorders
Idiopathic Pulmonary Fibrosis
Introduction
Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, causes progressive pulmonary fibrosis. Symptoms and signs develop over months to years and include exertional dyspnea, cough, and fine (Velcro) crackles. Diagnosis is based on history, physical examination, high-resolution CT, and/or lung biopsy, if necessary. Treatment may include antifibrotic drugs and oxygen therapy. Most patients deteriorate; median survival is about 3 years from diagnosis. Idiopathic pulmonary fibrosis, identified histologically as usual interstitial pneumonia, accounts for most cases of Overview of Idiopathic Interstitial Pneumonias . IPF affects men and women > 50 in a ratio of 2:1, with a markedly increased incidence with each decade of age. Current or former cigarette smoking is most strongly associated with the disorder. There is some genetic predisposition; familial clustering occurs in up to 20% of cases. 