Journal of Cardiovascular Development and Disease
Pulmonary Veno-Occlusive Disease in Systemic Sclerosis: A Scoping Review of the Literature
By Francesco Carta, Beatrice Moccaldi, Giulia Famoso and 8 others.
Abstract
Background
Patients with systemic sclerosis (SSc) may be affected by pulmonary veno-occlusive disease (PVOD), a poorly characterized form of group I pulmonary arterial hypertension (PAH). This scoping review aimed to systematically map the literature regarding SSc-PVOD to identify key clinical features, knowledge gaps, and future research areas.
Methods
A systematic literature search was conducted (January 1970 to May 2026) across PubMed, Scopus, and Cochrane databases following the PRISMA-ScR guidelines. We included adult patients with right heart catheterization (RHC)-confirmed SSc-PAH and a concurrent/subsequent diagnosis of PVOD established via pulmonary edema following vasodilators, highly suggestive chest HRCT findings, or histopathology.