ArticleInternal medicine (Tokyo, Japan)2026
Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis.
Article in Internal medicine (Tokyo, Japan), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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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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