Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis
Abstract
Occasionally, sarcoidosis can progress to fibrotic lung disease. Pleuroparenchymal fibroelastosis (PPFE) is a rare fibrotic pattern characterized by upper lobe-predominant pleural and subpleural fibroelastoses. However, pathologically confirmed cases of PPFE in sarcoidosis patients have not been reported. We herein describe the case of a 59-year-old woman with long-standing pulmonary sarcoidosis who developed progressive upper lobe-predominant fibrosis and cystic changes, culminating in living-donor lung transplantation. Histopathological examination of the explanted lungs revealed pleural thickening and subpleural intra-alveolar fibroelastosis, consistent with PPFE in the absence of typical granulomatous lesions of sarcoidosis. This case provides the first pathological evidence that PPFE represents a fibrotic manifestation of end-stage pulmonary sarcoidosis.
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BibTeX
@article{kono2026pleuroparenchymal,
title = {{Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis}},
author = {Masato Kono and Takahiko Saito and Takuma Sugiura and Misato Higasa and Fumiya Nihashi and Yuya Aono and Mineo Katsumata and Hideki Miwa and Yoshihiro Miki and Dai Hashimoto and Satona Tanaka and Hiroshi Date and Takafumi Suda and Hidenori Nakamura},
journal = {Internal Medicine},
year = {2026},
month = sep,
doi = {10.2169/internalmedicine.6496-25},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/65/17/65_6496-25/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis
AU - Masato Kono
AU - Takahiko Saito
AU - Takuma Sugiura
AU - Misato Higasa
AU - Fumiya Nihashi
AU - Yuya Aono
AU - Mineo Katsumata
AU - Hideki Miwa
AU - Yoshihiro Miki
AU - Dai Hashimoto
AU - Satona Tanaka
AU - Hiroshi Date
AU - Takafumi Suda
AU - Hidenori Nakamura
PY - 2026
DA - 2026/09/01
JO - Internal Medicine
DO - 10.2169/internalmedicine.6496-25
UR - https://www.jstage.jst.go.jp/article/internalmedicine/65/17/65_6496-25/_article
AB - Occasionally, sarcoidosis can progress to fibrotic lung disease. Pleuroparenchymal fibroelastosis (PPFE) is a rare fibrotic pattern characterized by upper lobe-predominant pleural and subpleural fibroelastoses. However, pathologically confirmed cases of PPFE in sarcoidosis patients have not been reported. We herein describe the case of a 59-year-old woman with long-standing pulmonary sarcoidosis who developed progressive upper lobe-predominant fibrosis and cystic changes, culminating in living-donor lung transplantation. Histopathological examination of the explanted lungs revealed pleural thickening and subpleural intra-alveolar fibroelastosis, consistent with PPFE in the absence of typical granulomatous lesions of sarcoidosis. This case provides the first pathological evidence that PPFE represents a fibrotic manifestation of end-stage pulmonary sarcoidosis.
SN - 1349-7235
SN - 0918-2918
ER -