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Updated: Jul 20, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension
Sindhu R Johnson1, Demetris Patsios, David M Hwang
1Division of Rheumatology, Department of Pathology, University Health Network, University of Toronto, Toronto, Ontario, Canada.
Objective:
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presumed PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect.
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