[Idiopathic pulmonary fibrosis]

Abstract

Idiopathic pulmonary fibrosis (IPF), known in Europe as cryptogenic fibrosing alveolitis, is a rare, progressive and usually fatal form of idiopathic interstitial pneumonia. IPF is characterized by failure of alveolar re-epithelization, persistence of fibroblasts, deposition of extracellular matrix, and distortion of lung architecture which ultimately results in respiratory failure. Current consensus statements reserve the term IPF to refer to a specific clinical entity associated with the histopatological pattern of usual interstitial pneumonia (UIP). UIP is characterized by temporal heterogeneity, with alternating areas of interstitial fibrosis, fibroblastic foci (areas of proliferating fibroblasts and myofibroblasts), inflammation, honeycomb lung, and normal parenchyma. Fibroblastic foci are associated with progressive disease. Treatment of IPF remains clinical problem. Currently, there is no conservative therapy improving the survival of patients. Lung transplantation, however, improves survival. Identification of pathways crucial to fibrogenesis might offer potentially novel therapeutic targets to slow or halt progression of IPF.