Fibroelastosis pleuropulmonar idiopática

Abstract

BACKGROUND: we sought to review the diagnostic criteria and potential treatments in the idiopathic pleuroparenchymal fibroelastosis. MATERIAL AND METHODS: pleuroparenchymal fibroelastosis is a new medical entity recently described in the scientific literature, so we have chosen all kind of articles released in the last fifteen years, between 2000 and 2015. We have selected articles written in English about fibroelastosis described in human case reports, collecting seventeen studies, including direct and indirect search. RESULTS: pleuroparenchymal fibroelastosis is a rare condition characterised by markedly thickened visceral pleura and prominent subpleural fibrosis, predominantly in the upper lobes, with intra-alveolar fibrosis and alveolar septal elastosis. The most common presenting complaints were dyspnoea and cough, with a restrictive ventilatory pattern. There is currently no specific therapy, only supportive treatment and lung transplantion are useful in this pathology. This illness often runs a rapidly progressive functional deterioration even in a relatively short time of follow-up with a poor prognosis, therefore life expectancy is lower than seven years. CONCLUSIONS: Although there have been described many hypothesis about the pathogenesis of this disease, it is still unknown, so that this lack of knowlege makes it difficult to find effective treatments which could inmprove life expentancey and quality of life of these patients.