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Clinical and genetic factors predictive of mortality in early systemic sclerosis
Arthritis Care and Research , 09/30/09

Assassi Set al. – A limited number of variables collected at presentation, including BMI, are able to predict mortality in patients with early SSc. In addition, some of the HLA genes associated with SSc susceptibility are useful for predicting SSc outcome.

Methods

250 patients
In addition to clinical and laboratory data, electrocardiograms (EKGs), chest radiographs, and pulmonary function tests have been obtained from each patient
A modified Rodnan skin thickness score, HLA class II genotyping, and a Medsger Damage Index also have been collected

Results

The study analyzed 122 white, 47 African American, and 71 Hispanic SSc patients with an average disease duration of 2.6 years at enrollment
At the time of analysis, 52 (20.8%) of the 250 patients had died
In the final multivariable model excluding HLA genes, 7 variables emerged as significant predictors of mortality: age 65 years at enrollment, forced vital capacity <50% predicted, clinically significant arrhythmia on EKG, absence of anticentromere antibodies, hypertension, chest radiograph suggestive of pulmonary fibrosis, and low body mass index (BMI)
In separate modeling that included HLA genes, HLA alleles DRB1*0802 and DQA1*0501 were significant predictors of mortality in addition to the predictors mentioned above

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