Published: 12 June 2020
Author(s): Cosimo Bruni, Giacomo De Luca, Maria-Grazia Lazzaroni, Elisabetta Zanatta, Gemma Lepri, Paolo Airò, Lorenzo Dagna, Andrea Doria, Marco Matucci-Cerinic
Issue: August 2020

Pulmonary arterial hypertension (PAH) is a rare and severe disease characterized by proliferative remodeling of the small pulmonary arteries, leading to increased pulmonary vascular resistance (PVR), ultimately resulting in right heart failure and death. In Systemic Sclerosis (SSc), PAH remains one of the main determinants of mortality in about 8–12% of patients [1,2]. Several studies conducted on large cohorts have highlighted that all SSc patients carry a certain risk, although variable, to develop PAH [3].


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