Open Access Rheumatology: Research and Reviews (Dec 2019)
Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
Abstract
Didem Saygin,1 Robyn T Domsic2 1Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; 2Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USACorrespondence: Robyn T DomsicDivision of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, S706 BST, 200 Lothrop Street, Pittsburgh, PA 15213, USATel +1 412 383 8000Fax +1 412 383 8765Email [email protected]: Systemic sclerosis (SSc) is a chronic, multisystem autoimmune disease characterized by vasculopathy, fibrosis and immune system activation. Pulmonary hypertension and interstitial lung disease account for majority of SSc-related deaths. Diagnosis of SSc-PAH can be challenging due to nonspecific clinical presentation which can lead to delayed diagnosis. Many screening algorithms have been developed to detect SSc-associated pulmonary arterial hypertension (SSc-PAH) in early stages. Currently used PAH-specific medications are largely extrapolated from IPAH studies due to smaller number of patients with SSc-PAH. In this review, we discuss the current state of knowledge in epidemiology and risk factors for development of SSc-PAH, and challenges and potential solutions in the diagnosis, screening and management of SSc-PAH.Keywords: scleroderma, pulmonary hypertension, screening
