Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients

Abstract

BackgroundIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported group of disorders characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes. We report five Japanese cases fulfilling the criteria of IPPFE and address whether it should be considered a separate clinicopathologic entity. And this study was an attempt to identify features in common between IPPFE and previously described idiopathic upper lobe fibrosis (IPUF), allowing IPPFE to be considered as a distinct entity in our Japanese series.MethodsFive consecutive cases of idiopathic interstitial lung disease confirmed as IPPFE by surgical lung biopsy were studied.ResultsThere were four males and one female, aged 70±2.76 yr. No associated disorder or presumed cause was found in any case. Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5). Chest X-ray showed marked apical pleural thickening in all cases. Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes. In all cases in this study, markedly thickened visceral pleura and prominent subpleural fibrosis characterized by both elastic tissue and dense collagen were clearly shown. All cases were alive at the last follow-up, 17.6±13.59 months after diagnosis; however, all had deteriorated both clinically and radiologically.ConclusionsIPPFE deserves to be defined as a separate, original clinicopathologic entity owing to its uniformity and IPPFE has some features in common with previously described idiopathic upper lobe fibrosis (IPUF). Our limited experience with a cohort of 5 subjects suggests that IPPFE can be rapidly progressive.

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

“…The clinical course of this disease is progressive and almost all patients clinically and functionally deteriorate even over a relatively short follow-up period. Moreover, the prognosis of IPPFE patients has been reported to be poor (5).…”

“…Several reports of idiopathic upper lobe fibrosis (IPUF) have been published in the Japanese literature since 1992 (2)(3)(4), and IPUF and IPPFE may belong to the same disease entity. While the clinical features of IPPFE are not entirely clear because of its extreme rarity, the condition may be divisible into subgroups with or without lesions of usual interstitial pneumonia (UIP) with fibroblastic foci in the lower lobes (5). The clinical course is considered progressive, and almost all patients clinically and functionally deteriorate even over a relatively short follow-up period (5,6).…”

“…While the clinical features of IPPFE are not entirely clear because of its extreme rarity, the condition may be divisible into subgroups with or without lesions of usual interstitial pneumonia (UIP) with fibroblastic foci in the lower lobes (5). The clinical course is considered progressive, and almost all patients clinically and functionally deteriorate even over a relatively short follow-up period (5,6). The prognosis has been reported to be poor, and there are no established therapeutic options available for IPPFE except for supportive care and, ultimately, lung transplantation (7).…”

Adolescente con fibroelastosis pleuroparenquimatosa idiopática. A propósito de un caso

“…En el 32% de los pacientes con FEPPI se observaron antecedentes de tumores malignos. 6 También se ha asociado la FEPPI con quimioterapia y radioterapia. Los trasplantes de médula ósea o de pulmón podrían causar FEPPI, como una manifestación pulmonar de enfermedad injerto contra huésped crónica.…”

An adolescent with idiopathic pleuroparenchymal fibroelastosis. Case report

“…History of malignancy was found to be 32% in IPPFE. 6 Chemotherapy and radiotherapy is also associated with IPPFE. Bone marrow and lung transplantation may cause IPPFE, as a lung manifestation of chronic graft versus host disease.…”