Presentation: Fulminant Scleroderma as an Early Cause of Death in Patients with Systemic Sclerosis (2007)

20 Fulminant Scleroderma as an Early Cause of Death in Patients with Systemic Sclerosis

Objective: To compare the characteristics of patients with systemic sclerosis who died within 2 years of diagnosis to those who died after 3 years of diagnosis.
Methods: Review of all medical records of patients with systemic sclerosis followed at the Scleroderma Center of our institution who died during a 20 year period (1987-2006) and comparison of clinical and laboratory test results between those with early mortality (2 years or less from diagnosis) and those with late mortality (3 years or more from diagnosis).
Results: We identified 20 patients with early mortality and 67 with late mortality. There were no statistically significant differences between the two groups regarding gender, age, race, clinical subset of systemic sclerosis, ANA presence and pattern, ScL-70 antibodies, initial DLCO measurement and initial pulmonary artery pressure. Patients with early mortality were more likely to be anticentromere negative as compared to the patients who died after 3 years (17/20 vs 48/67, p=0.03). The interval from initial symptoms of Raynaud's phenomenon to diagnosis was similar in both groups, whereas the interval from the first appearance of non-Raynaud's symptoms to diagnosis was significantly shorter in the early mortality group than in the late mortality group (11.8 ± 10.3 vs 60.7 ± 64.9 months, p=0.002). According to Medsger's 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 initial evaluation in patients with early mortality as compared to the late mortality group. The time from the first non Raynaud's symptom to initiation of disease modifying treatment was significantly shorter in early mortality patients (9.43 ±6.3 vs 38.3 ±54.4 months, p=0.05). The time of treatment initiation to death was also shorter in the early mortality group (15.1±9.48 vs 60.7±49.7 months, p=0.001), reflecting a much more severe disease. Causes of death in the early mortality group were: myocardial infarction in 8, acute cardiopulmonary failure in 5, and pulmonary hypertension in 2 patients. Respiratory failure and sudden death were predominant causes of mortality in patients who died after 3 years of diagnosis. Conclusion: Patients who died within the first 2 years of diagnosis of systemic sclerosis were typically anticentromere negative, had significant muscle and cardiac involvement. The time of first appearance of non-Raynaud’s symptoms was much shorter and disease modifying treatment was instituted earlier in patients who died within 2 years of diagnosis suggesting a very fulminant form of systemic sclerosis in this group.
Supported by NIH Grants T32AR007583 (GH) and R01AR19616

 G. Huaman, None; F. Otieno, None; S.A. Jimenez, None; C.T. Derk, None.