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

• Main Authors: Ţilea Ioan, Bocicor Elena Andreea, Gal Codruţa Maria, Varga Andreea
• Format: Article
• Language: English
• Published: Sciendo 2016-06-01
• Series: Journal of Interdisciplinary Medicine
• Subjects: pulmonary hypertension; connective tissue disease; diagnosis; specific therapy; multidisciplinarity
• Online Access: https://doi.org/10.1515/jim-2016-0019

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.