To compare the characteristics of patients with systemic sclerosis who died within 2 years of diagnosis to those who died after 2 years of diagnosis. A retrospective chart review of all medical records of deceased systemic sclerosis (SSc) patients who had been followed at our institution from 1985 to 2007 was performed. We identified 87 deceased SSc patients within this period. From the 87 deceased individuals, 20 had died within 2 years after they were diagnosed, and 67 had died after 2 years of their diagnosis. Patients who died within 2 years of diagnosis were more likely to be anticentromere antibody negative when compared to the patients who died after 2 years (17/20 vs. 48/67, P = 0.03). The time from the first appearance of non-Raynaud's symptoms to diagnosis was significantly shorter in the group who died within 2 years than in the group who died after 2 years of diagnosis (11.8 ± 10.3 vs. 60.7 ± 64.9 months, P = 0.002). According to the Medsger severity score, there was more severe muscle (0.82 ± 1.13 vs. 1.8 ± 1.28, P = 0.0014) and heart (0.86 ± 1.37 vs. 2.1 ± 1.71, P = 0.0013) involvement at the initial evaluation in patients who died before 2 years of diagnosis when compared to the group of patients who died after 2 years of diagnosis. The time from the first symptoms to treatment initiation was significantly shorter in patients who died early (9.43 ± 6.3 vs. 38.3 ± 54.4 months, P = 0.05). The interval between treatment initiation and death was also significantly shorter (15.1 ± 9.48 vs. 60.7 ± 49.7 months, P = 0.001), reflecting greater severity of disease. Patients who died within the first 2 years of SSc diagnosis were typically anticentromere negative and had significant muscle and cardiac involvement. The time from the first appearance of non-Raynaud phenomenon symptoms to death was much shorter in the patients who died within 2 years of diagnosis, suggesting a very fulminant form of systemic sclerosis.