Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis

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.