OBJECTIVES Data on the role of tobacco exposure in systemic sclerosis (SSc) severity and progression are scarce. We aimed to assess the effects of smoking on the evolution of pulmonary and skin manifestations in the EUSTAR database. METHODS Adult SSc patients with data on smoking history and a 12-24 months follow-up visit were included. Associations of severity and progression of organ involvement with smoking history and the comprehensive smoking index (CSI) were assessed using multivariable regression analyses. RESULTS 3,319 patients were included (age 57 years; 85% female), 66% were never smokers; 23% ex-smokers and 11% were current smokers. Current smokers had a lower percentage of anti-topoisomerase autoantibodies than previous or never smokers (31% vs. 40% and 45%, respectively). Never smokers had a higher baseline forced expiratory volume in one second/forced vital capacity (FEV1/FVC) ratio than previous and current smokers (p<0.001). The FEV1/FVC ratio declined faster in current smokers than in never smokers (p=0.05) or ex-smokers (p=0.01). The baseline modified Rodnan skin score (mRSS) and the mRSS decline were comparable across smoking groups. Although heavy smoking (more than 25 pack years) increased the odds of digital ulcers by almost 50%, there was no robust adverse association of smoking with digital ulcer development. CONCLUSION The known adverse effect of smoking on bronchial airways and alveoli is also observed in SSc patients; however robust adverse effects of smoking on the progression of SSc-specific pulmonary or cutaneous manifestations were not observed. This article is protected by copyright. All rights reserved.