Risk of Digital Vascular Events in Scleroderma Patients Who Have Both Anticentromere and Anti-Interferon-Inducible Protein 16 Antibodies.

OBJECTIVE: To evaluate whether scleroderma patients who are double-positive for anti-interferon-inducible protein 16 (anti-IFI-16) antibodies and anticentromere (anti-CENP) antibodies are at increased risk for significant digital vascular events relative to patients positive for anti-CENP antibodies alone. METHODS: Sera from 165 scleroderma patients who tested positive for anti-CENP antibodies upon clinical evaluation were reassayed for both anti-CENP and anti-IFI-16 antibodies using enzyme-linked immunosorbent assay testing. 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-square tests. After completion of univariate analysis, multivariable analyses were done to adjust for clinically relevant covariates. RESULTS: 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 adjustment for clinically relevant covariates (age, cutaneous subtype, disease duration, and smoking), double-positive patients remained at significantly higher odds of having severe Raynaud's phenomenon (odds ratio 3.5 [95% confidence interval 1.1-11.1]; P = 0.03). CONCLUSION: 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.

New clinical and ultrastructural findings in hydroxychloroquine-induced cardiomyopathy--a report of 2 cases.

Hydroxychloroquine- or chloroquine -induced cardiomyopathy is a rare but potentially fatal condition. Hydroxychloroquine and chloroquine are often used for long-term treatment of rheumatic diseases and for malaria prophylaxis. Hydroxychloroquine- and chloroquine-induced cardiomyopathy have well-described microscopic features, with the classic electron microscopic findings of myelin figures (myeloid bodies). We report on 2 new cases with novel findings. The first case, in a patient with systemic lupus erythematosus, was found to have megamitochondria in addition to myelin figures seen by electron microscopy. The second report describes the first case of hydroxychloroquine cardiomyopathy described in a patient with scleroderma. These novel findings will add to the present knowledge of hydroxychloroquine-induced cardiomyopathy in its pathology and its implication for treatment of rheumatic diseases.