Pleuroparenchymal fibroelastosis - clinical analysis of 22 cases

Abstract

Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a rare disease, recently described and characterized by fibroelastotic thickening of the pleural and subpleural lung parenchyma, mainly in upper lobes. There is a raising number of diagnosis, but the etiology and pathophysiology are still unknown. Objectives: The objectives of this study were to identify and characterize the patients with PPFE, followed in Hospital de Sao Joao, a hospital university center. Methods: It was made a retrospective analysis of patients with criteria of PPFE. We analyzed demographic, clinical parameters including smoke status, initial symptoms, respiratory functional data and treatment. Results: It was found 22 cases with PPFE diagnostics criteria. The mean age was 62.3 years old. Most patients were woman (n=16) and non-smokers (n=14). Most common symptom at the time of diagnosis was dyspnea, followed by cough, weight loss, repetitive respiratory infections, wheezing and pleuritic pain. In functional evaluation, 12 patients had reduced diffusing capacity; 4 restrictive pattern and 2 obstructive pattern. PPFE was considered idiopathic in 8 patients and was associated with usual interstitial pneumoniae (n=5), chronic hypersensitivity pneumonitis (n=2), exuberant emphysema (n=2), pulmonary cancer (n=2), silicosis (n=1), bronchiectasis (n=1) and amiodarone toxicity (n=1). Only 10 patients were on immunosuppressive treatment. Discussion: The evaluation of this patients confirmed that PPFE has a non-specific and heterogeneous nature, given the different associations and contexts in which occurs. Then, it´s crucial to study the idiopathic form, that can more accurately evidence the natural evolution of this disease and response to treatment.