Idiopathic pulmonary fibrosis - clinical presentation,outcome and baseline prognostic factors in a Portuguese cohort [Fibrose pulmonar idiopática: Apresentação clínica,evolução e fatores de prognóstico basais numa coorte portuguesa]

Introduction: idiopathic pulmonary fibrosis (ipf) is the most common disease in the subgroup of idiopathic interstitial pneumonias. it is inevitably associated to a bad prognosis,although assuming a highly variable clinical course. methods: patients with ipf,observed at interstitial lung diseases outpatient clinic of centro hospitalar de são joão - porto,portugal,were identified and clinical,functional,radiological and bronchoalveolar lavage (bal) parameters were reviewed. their clinical course and survival were analyzed in order to identify prognostic factors. results: eighty-one patients were included,with a mean age at diagnosis of 63.8 years old. at diagnosis,the main functional abnormalities were restrictive physiology,reduced lung diffusion and exercise capacity impairment. clinical course was mainly slowly progressive (72.3%). ten patients (13.2%) had a rapid progression and 11 (14.5%) patients had an acute exacerbation during the course of the disease. ipf's rapid progression was associated to a higher functional impairment at diagnosis,namely in what is related with forced vital capacity (fvc) and total lung capacity (tlc). median survival was 36 months. a significant difference in survival was observed among different types of clinical course - 41 months for slow progressors and 9 months for rapid progressors. lower levels of fvc,tlc,6th minute walk test distance and rest pao2,and higher bal neutrophil count were associated with poorer survival in univariate analysis. conclusion: the analysis of this group of ipf patients confirms two clearly different phenotypes,slow and rapid progressors. those phenotypes seem to have different presentations and a remarkably different natural history. these results could mean different physiopathologic pathways,which could implicate different therapeutic approaches. © 2011 sociedade portuguesa de pneumologia.