Limited Cutaneous Scleroderma-Related Pulmonary Arterial Hypertension — a Case Report

Abstract

Background: Pulmonary hypertension (PH), defined by mean pulmonary arterial pressure (PAPm) ≥25 mm Hg, can lead to increasing pulmonary vascular resistance, which eventually results in right ventricle failure. Scleroderma, as an autoimmune connective tissue disease, is associated with PH as a sub-group according to the 2015 ESC/ERS PH classification. Pulmonary arterial hypertension (PAH) associated with scleroderma (SSc-PAH) can often result in poor prognosis with increased mortality. Early diagnosis and specific treatment of PH can significantly improve the prognosis of these patients.

Keywords

• pulmonary hypertension
• connective tissue disease
• diagnosis
• specific therapy
• multidisciplinarity