BackgroundDespite the high incidence of sarcopenia in systemic sclerosis (SSc) patients, there is currently limited evidence on their outcomes. ObjectivesOur study aimed to determine clinical courses, outcomes, and identify factors associated with mortality in the SSc patients with sarcopenia. MethodsA historical cohort study was conducted in 180 adult SSc patients diagnosed with sarcopenia according to the criteria of Asian Working Group for Sarcopenia 2019, who were attending the Scleroderma Clinic at Khon Kaen University between July 2019 and November 2021. ResultsForty-one were diagnosed with sarcopenia. A total of 443.8 persons-year, the respective mortality rate for SSc patients with and without sarcopenia was 5.05 and 5.22 per 100-person-years, showing no statistical difference (p = 0.58). Sarcopenia was not a significant mortality risk in SSc patients with a hazard ratio (HR) of 1.34, 95 % CI 0.48–3.75. The survival rate from the baseline evaluation of sarcopenia to the last follow-up of the patients with sarcopenia at 6-, 12-, 18-, and 24-months were 97.6 %, 95.1 %, 92.7 %, and 87.8 %. Hospitalization was the sole factor significantly associated with the mortality risk, with a HR of 14.21 (95 % CI 2.36–85.60). Sarcopenia itself did not appear to be a significant predictor of disease progression, it did contribute significantly to the progression of salt and pepper skin (p=0.01). ConclusionsThe mortality rate of SSc patients with sarcopenia increased after a 2-year follow-up but no difference from non-sarcopenic patients. Once these patients required hospitalization, the mortality risk increased by over 10 times. Further long-term follow-up in a large cohort is suggested.