Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis.
Masato Kono, Takahiko Saito, Takuma Sugiura et al.
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Case in brief
A 59-year-old woman with long-standing pulmonary sarcoidosis developed progressive upper-lobe-predominant fibrosis and cystic changes, ultimately undergoing living-donor lung transplantation. Examination of the explanted lungs showed pleural thickening and subpleural intra-alveolar fibroelastosis consistent with pleuroparenchymal fibroelastosis (PPFE). Typical granulomatous lesions of sarcoidosis were absent in the examined tissue.
What made this case unusual
Histopathology identified PPFE in a patient with fibrotic pulmonary sarcoidosis, despite the absence of typical granulomatous lesions in the explanted lungs.
Diagnostic / clinical pearl
In progressive upper-lobe-predominant fibrosis associated with sarcoidosis, explant histopathology may reveal PPFE even when typical granulomas are absent.
Why it matters
The case provides pathological evidence supporting PPFE as a possible fibrotic manifestation of end-stage pulmonary sarcoidosis.