Fibrosis pulmonar idiopática

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic idiopathic disease differentiated, progressive fibrosing interstitial pneumonia. It usually affects adults over 50 years old and presents with progressive exertional dyspnea and dry cough. Prevalence rales were heard at bases in 90 % of patients and there clubbing in 50 %. Definitive diagnosis requires: a) the exclusion of other clinical entities defined or diffuse lung diseases of known cause; b) radiological evidence of a typical pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT), the presence of a histological pattern of UIP on lung biopsy specimens adequate or both. The diagnostic accuracy improves with multidisciplinary assessment among clinicians, radiologists and pathologists expert. A better understanding of the pathogenesis of IPF has helped to launch several clinical trials in search of more selective and effective to the poor prognosis of the disease therapeutic strategies.