Idiopathic pleuroparenchymal fibroelastosis: first case report in Lebanon

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was initially described by Amitani et al. in Japan. It is characterized by visceral pleural fibrosis and adjacent lung parenchymalfibroelastosis with striking upper lobe predominance. Because of its rarity and the lack of clear diagnostic criteria, the prevalence of the disease is still unclear. We report the first case of IPPFE in Lebanon and the second one in the Arab World. A 37-year-old Iraqi man was admitted to the hospital with progressive dyspnea on exertion occurring since 2 years and associated with dry cough. Histo-pathologic results reported a prominent interstitial fibrosis mainly in upper parts, and no granulomatous tissue was detected. Therefore, the diagnosis of IPPFE was made. The IPPFE is a distinct entity that requires meticulous clinico-pathological correlation for an adequate diagnosis and a close follow-up as this entity can progress into more advanced stages.