CLINICAL THERAPEUTICS AND HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCuneManagement of systemic sclerosis the first five yearsRoofeh, David; Khanna, DineshAuthor Information Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA Correspondence to David Roofeh, MD, Department of Internal Medicine, Division of Rheumatology, 300 North Ingalls St., Suite 7C13, Ann Arbor, MI 48109-5422, USA. Tel: +1 734 936 5561; fax: +1 734 936 3695; e-mail: email@example.com Current Opinion in Rheumatology: May 2020 - Volume 32 - Issue 3 - p 228-237 doi: 10.1097/BOR.0000000000000711 Buy Metrics Abstract Purpose of review This review provides a risk-stratified and evidence-based management for subsets of systemic sclerosis (SSc) patients in the first five years from disease onset. Recent findings Cardiopulmonary disease remains the primary cause of mortality in SSc patients. Morbidity and mortality in SSc-associated pulmonary arterial hypertension have improved with combination treatment, in either an upfront or sequential treatment pattern. Traditional therapies for interstitial lung disease (SSc-ILD) have targeted those with clinically significant and progressive ILD with immunosuppression. New data suggest a possible paradigm shift, introducing immunosuppressive therapy to patients before they develop clinically significant or progressive ILD. The year 2019 saw the approval of the first FDA-approved therapy for SSc-associated interstitial lung disease, using an antifibrotic agent previously approved for idiopathic pulmonary fibrosis. To date, only autologous hematopoietic stem cell transplant has demonstrated a mortality benefit for SSc-ILD, albeit in a narrow spectrum of SSc-ILD patients. Summary SSc is a highly heterogeneous autoimmune disease typified by varying clinical trajectories. Its management may be stratified within the first five years by subclassifying patients based on factors that have important prognostic significance: skin distribution and autoantibody status. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.