Pleuroparenchymal fibroelastosis – a new diagnostic and therapeutic challenge for pneumologists

Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a rare condition with upper lobe predominant fibroelastosis and pleural thickening. PPFE is now recognized as specific rare entity, usually idiopathic, and included as a new entity in the updated classification of the idiopathic interstitial pneumonia. Case Report: A 63 years old non-smoking woman presented with progressive dyspnoea on exertion and dry cough. There was no significant occupational exposure, allergy or history of systemic disease. 3 years ago idiopathic pulmonary fibrosis (IPF) was diagnosed by surgical biopsy in another hospital. At current…