Pleuroparenchymal Fibroelastosis of the Lung: A Review.

Pleuroparenchymal Fibroelastosis of the Lung: A Review.

Arch Pathol Lab Med. 2016 Aug;140(8):849-853

Authors: Cheng SK, Chuah KL

Abstract
Described in Japan by Amitani et al in 1992, the entity of idiopathic upper lobe fibrosis was subsequently given the name pleuroparenchymal fibroelastosis (PPFE) in the English-speaking world. Pleuroparenchymal fibroelastosis is believed to be a rare disease characterized by a fibrosing process affecting the pleura and the subpleural lung parenchyma, with a predilection for the upper lobes. Uniquely, the fibrosing process is elastotic in nature, being associated with intra-alveolar fibrosis. The etiology of PPFE is unclear at this juncture, with many cases being considered as idiopathic forms of the disease. Conditions associated with PPFE include infections, bone marrow transplantation, and autoimmunity. In this review, we explore the clinical, radiologic, and pathologic features associated with PPFE in light of current understanding of the disease. Recent studies implicated that PPFE may not be as uncommon as claimed. The various differential diagnoses and implications of diagnosing PPFE are discussed.

PMID: 27472241 [PubMed - as supplied by publisher]

from Head and Neck on PubMed via xlomafota13 on Inoreader http://ift.tt/2ah1JvV
via IFTTT

http://ift.tt/2ajU04z