Increased risk of digital vascular events in scleroderma patients who have both anti-centromere and anti-interferon-inducible protein 16 antibodies.

We sought to evaluate whether scleroderma patients who are double-positive for anti-interferon inducible protein 16 (IFI-16) antibodies and anti-centromere (CENP) antibodies are at increased risk for significant digital vascular events relative to patients positive for anti-CENP antibodies alone.,Sera from 165 scleroderma patients who tested positive for anti-CENP antibodies upon clinical evaluation were re-assayed for both anti-CENP and anti-IFI-16 antibodies by ELISA. Patients who were positive for anti-CENP antibodies alone were then compared to patients who were double-positive for both anti-IFI-16 and anti-CENP antibodies. The association between a history of significant digital vascular events (digital pits, ischemic digital ulcers, and/or gangrene) and double-positive antibody status was examined using Chi squared tests. After completion of univariate analysis, multivariable analyses were done to adjust for clinically relevant covariates.,Of the 165 anti-CENP antibody positive patients, 21 (12.7%) also had anti-IFI-16 antibodies. Patients who were double-positive for anti-CENP and anti-IFI-16 antibodies were more likely to have had digital pits, ischemic digital ulcers, and/or gangrene (p= 0.03). After adjusting for clinically relevant covariates, (age, cutaneous subtype, disease duration, and smoking), double-positive patients remained at significantly higher odds of having severe Raynaud's (OR = 3.5; CI 1.1, 11.1; p = 0.03).,Scleroderma patients who are double-positive for antibodies recognizing CENP and IFI-16 are significantly more likely to have significant digital vascular events during the course of their disease. This study provides further evidence that anti-CENP and anti-IFI-16 antibodies are disease biomarkers that may be used for risk stratification of vascular events in scleroderma. This article is protected by copyright. All rights reserved.