Over the past 30 years, survival rates for patients with systemic sclerosis (SSc) have improved significantly, with 10-year survival increasing from 54% to 66%. The primary causes of death have shifted from renal crisis to pulmonary complications, including pulmonary arterial hypertension (PAH) and pulmonary fibrosis (PF). Renal crisis-related deaths decreased from 42% to 6% of scleroderma-related deaths, while PF-related deaths increased from 6% to 33%. PAH-related deaths also increased. Scleroderma-related deaths decreased from 70% to 50% of all deaths. Non-scleroderma-related causes, such as cancer and cardiovascular disease, accounted for 50% of deaths, with no significant changes in their distribution. The study highlights the importance of continued research into therapies for severe pulmonary complications of SSc. The survival of patients with SSc has improved over the past 30 years, with lung disease now being the primary cause of death. The study was conducted at the University of Pittsburgh and involved a cohort of patients followed over a 25-year period. The results show that improved management and treatment of SSc have led to better survival rates, despite the continued presence of severe pulmonary complications. The study also found that patients with diffuse SSc had improved survival compared to those with limited SSc. The study was funded by the Scleroderma Foundation. No competing interests were declared.Over the past 30 years, survival rates for patients with systemic sclerosis (SSc) have improved significantly, with 10-year survival increasing from 54% to 66%. The primary causes of death have shifted from renal crisis to pulmonary complications, including pulmonary arterial hypertension (PAH) and pulmonary fibrosis (PF). Renal crisis-related deaths decreased from 42% to 6% of scleroderma-related deaths, while PF-related deaths increased from 6% to 33%. PAH-related deaths also increased. Scleroderma-related deaths decreased from 70% to 50% of all deaths. Non-scleroderma-related causes, such as cancer and cardiovascular disease, accounted for 50% of deaths, with no significant changes in their distribution. The study highlights the importance of continued research into therapies for severe pulmonary complications of SSc. The survival of patients with SSc has improved over the past 30 years, with lung disease now being the primary cause of death. The study was conducted at the University of Pittsburgh and involved a cohort of patients followed over a 25-year period. The results show that improved management and treatment of SSc have led to better survival rates, despite the continued presence of severe pulmonary complications. The study also found that patients with diffuse SSc had improved survival compared to those with limited SSc. The study was funded by the Scleroderma Foundation. No competing interests were declared.