Fibroélastose pleuro-parenchymateuse

Abstract

Idiopathic pleuroparechymal fibroelastosis appears in the litlerature under that name in 2004.The diagnosis is usually suspected on the basis of a patient with dyspnea and a compatible radiographic pattern with a pleural thickening as well as a subpleural fibrosis in the uppers lobes and a decreasing pulmonary volume, an ascension of the pulmonary hiliums and a shrinking of the apex.The definitive diagnosis requires anatomopathological analysis with specific colouring of Elastin fibres.Differential diagnosis are tuberculosis consequences, connective tissue disease, polyarteritis, the lung asbestosis, and the apical cap.The disease can appear at any age, dyspnea is still present, caughs, weight loss and chest pain are common. Platythorax is typical. Lung fonction is altered with a restrictive spirometry pattern. Spontaneous pneumothorax is a common complication. The disease progresses toward a restrictive and an hypercanic respiratory insufficiency.Etiologies are idiopathic and commonly iatrogenic (lung transplantation, chemotherapy, bone marrow transplantation). There also are familial forms with genetics abnormalities.The prognosis is poor with a fast vital capacity decreasing.The corticosteroids are useless. At the moment there is no effective treatment other than lung transplantation.