Idiopathic Pleuroparenchymal Fibroelastosis

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•• Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012;40(2):377–85. This study provides the first extensive description of PPFE features, suggesting a list of radiological and histological criteria for diagnosis.

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Sato S, Hanibuchi M, Fukuya A, et al. Idiopathic pleuroparenchymal fibroelastosis is characterized by an elevated serum level of surfactant protein-D, but Not Krebs von den Lungen-6. Lung. 2014;192(5):711–7.

De Lauretis A, Basra H, Hakim W, et al. Pleuroparenchymal Fibroelastosis (PPFE) Predicts Survival in Idiopathic Pulmonary Fibrosis (IPF). 2016:A1142, 1110.1164/ajrccm-conference.2016.1193.1141_MeetingAbstracts.

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Yoshida Y, Nagata N, Tsuruta N, et al. Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. Respir Investig. 2016;54(3):162–9.

Enomoto N, Kusagaya H, Oyama Y, et al. Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF). BMC Pulm Med. 2014;14:91.

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Piciucchi S, Tomassetti S, Casoni G, et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 2011;12:111.

Sato S, Hanibuchi M, Takahashi M, et al. A patient with idiopathic pleuroparenchymal fibroelastosis showing a sustained pulmonary function due to treatment with pirfenidone. Intern Med. 2016;55(5):497–501.