Multivariate Model 1	Adjusted RR	95%CI	Multivariate Model 2	Adjusted RR	95%CI
Male vs Female	1.4	1.1 – 1.9	Male vs Female	1,4	1.1 – 1.9
African American vs Caucasian	1.5	1.1 – 2.1	African American vs Caucasian	1.3	1.0 – 1.8
Diffuse vs Limited	1.4	1.0 – 1.8	Diffuse vs Limited	1.2	0.9 – 1.5
Topoisomerase antibody, pos vs neg	1.0	0.7 – 1.3	Anti-centromere antibody, pos vs neg	0.8	0.6 – 1.0
Health Insurance, MA/no vs Mdcr/yes	1.2	0.8 – 1.7	Health Insurance, MA/no vs Mdcr/yes	1.4	1.0 – 1.8

Background/Purpose: The epidemiologic literature has identified several demographic, clinical and serologic predictors of mortality in systemic sclerosis (scleroderma). Yet, relatively few cohorts are of sufficient size and racial composition to examine the independent contribution of these parameters to survival. We sought to determine the adjusted risk of mortality in scleroderma associated with gender, race, disease subset, and serologic and socioeconomic status in a large observational cohort study.

Methods: Between January 1, 1990 and December 31, 2009, a total of 2217 patients with scleroderma, either African American (n=409, 18%) or Caucasian (n=1808, 82%), were evaluated at a single university medical center. The vital status of the cohort was ascertained using the Social Security Death Index; cumulative incidence of mortality was estimated using Kaplan-Meier analysis.  Next, the independent risk of mortality was estimated using Cox proportional hazards analysis, with adjustment for age at disease onset and disease duration, in addition to each variable depicted in the table below.

Results: This cohort of 2217 patients was 1838 (83%) female; mean age (+SD) at disease onset was 46 (+14) years. Among these patients, 1387 (63%) manifested the limited and 830 (37%) the diffuse cutaneous subtype of disease.  Overall, 1846 (83%) fulfilled ACR criteria for scleroderma, whereas 361 (16%) satisfied at least 3 of 5 CREST criteria. Among the 1511 patients whose sera was tested for anti-centromere, and 1393 assayed for anti-topoisomerase antibodies, 452 (30%) and 294 (21%) were seropositive, respectively. In addition, 11% of the cohort had medical assistance or no health insurance, whereas 89% had Medicare or private insurance.  During a median follow-up period of 4 years, 700 patients died. Overall, cumulative mortality at 1 and 5 years of follow-up was 8% and 32%, respectively. The relative risk of mortality, in two multivariate models, was as follows:

Conclusion: Persons with scleroderma who are male, African American, with diffuse cutaneous disease, and those without private or Medicare health insurance, experienced a heightened risk of mortality. Anti-centromere, but not topoisomerase, antibody was protective against mortality. These findings imply that demographic, clinical features, serologic and socioeconomic status, each impact upon and contribute to the risk of survival in scleroderma.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/demographic-clinical-serologic-and-socioeconomic-measures-each-predict-mortality-in-scleroderma/