Revista Portuguesa de Pneumologia Revista Portuguesa de Pneumologia
Rev Port Pneumol 2017;23:352-5 - Vol. 23 Num.6 DOI: 10.1016/j.rppnen.2017.06.006
Case report
Idiopathic pleuroparenchymal fibroelastosis (PPFE) – A case study of a rare entity
E.B. Boernera,, , U. Costabela, T.E. Wessendorfa, D. Theegartenb, F. Bonellaa
a Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg, Essen, Germany
b Department of Pathology, University Hospital Essen, University of Duisburg, Essen, Germany
Received 07 March 2017, Accepted 29 June 2017
Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was recognized as a rare new entity. We report the case of a 63 years old female suffering from progressive dyspnea and dry cough for three years. Two years before admission to our hospital, idiopathic pulmonary fibrosis (IPF) was diagnosed in another hospital and treatment with prednisolone and N-acetylcysteine (NAC) was commenced. At admission HRCT showed upper lobe dominant fibrosis and associated pleural thickening. Surgical biopsies were re-evaluated and revealed fibroelastosis with pleural thickening and a probable UIP pattern, consistent with idiopathic PPFE. Treatment with pirfenidone was initiated due to progression under prednisolone and NAC. Upper lobe predominant pleural thickening with associated subpleural fibrotic changes should raise suspicion of PPFE.

Keywords
Idiopathic pleuroparenchymal fibroelastosis, Pirfenidone, Rare lung disease
Rev Port Pneumol 2017;23:352-5 - Vol. 23 Num.6 DOI: 10.1016/j.rppnen.2017.06.006