Idiopathic Pulmonary Fibrosis – Diagnosis and Treatment

Abstract

Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively re- ferred to as interstitial lung diseases (ILDs) or diffuse paren- chymal lung diseases. Idiopathic pulmonary fibrosis (or cryp- togenic fibrosing alveolitis) (IPF or CFA) is one of several idiopathic interstitial pneumonias. IPF is now recognized as a distinct clinical disorder. Despite major accomplishments in our understanding of the pathogenesis of lung fibrosis (1), the diagnosis and management of patients with IPF continues to pose significant challenges (2–4).