Therapeutics and Clinical Risk Management (Dec 2019)
Therapeutic Challenges And Advances In The Management Of Systemic Sclerosis-Related Pulmonary Arterial Hypertension (SSc-PAH)
Abstract
Rahul G Argula,1 Celine Ward,2 Carol Feghali-Bostwick2 1Department of Medicine, Division of Pulmonary and Critical Care Medicine, Medical University of South Carolina, Charleston, SC 29425, USA; 2Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, SC 29425, USACorrespondence: Rahul G ArgulaDepartment of Medicine, Division of Pulmonary and Critical Care Medicine Medical University of South Carolina, 816 CSB, MSC 630, Jonathan Lucas Street, Charleston, SC 29425, USATel +1 843 792 3167Email [email protected]: Systemic sclerosis (SSc) is a rare autoimmune disorder with multi-organ involvement. SSc-associated pulmonary arterial hypertension (SSc-PAH) is one of the leading causes of morbidity and mortality in the SSc population. With advances in our understanding of pulmonary arterial hypertension (PAH) diagnosis and treatment, outcomes for all PAH patients have significantly improved. While SSc-PAH patients have also benefited from these advances, significant challenges remain. Diagnosis of PAH is a challenging endeavor in SSc patients who often have many co-existing pulmonary and cardiac comorbidities. Given the significantly elevated prevalence and lifetime risk of PAH in the SSc population, screening for SSc-PAH is a critically useful strategy. Treatment with pulmonary arterial (PA) vasodilators has resulted in a dramatic improvement in the survival and quality of life of PAH patients. While therapy with PA vasodilators is beneficial in SSc-PAH patients, therapy effects appear to be attenuated when compared to responses in patients with idiopathic PAH (IPAH). This review attempts to chronicle and summarize the advances in our understanding of the optimal screening strategies to identify PAH in patients with SSc. The article also reviews the advances in the therapeutic and risk stratification strategies for SSc-PAH patients.Keywords: systemic sclerosis, pulmonary arterial hypertension, screening, risk stratification, therapy, advances
