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Updated: Jan 22, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do?
Elena Bargagli1, Maria Antonietta Mazzei2, Martina Orlandi3
1Respiratory Diseases and Lung Transplantation Unit.
Abstract:
Pleuroparenchymal fibroelastosis (PPFE) is a rare new interstitial lung disease (ILD) characterized by the fibrotic thickening of the visceral pleura and subadjacent parenchymal areas of the upper lobes This study reveals that patients with ILD-SSc associated with chest HRCT evidence of PPFE require close and recurrent follow-up with periodic evaluation of lung function parameters, DLCO and chest HRCT. Rheumatologists should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention.
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