Authors:
Virginia Steen MD, Robyn T. Domsic MD MPH, Mary Lucas RN MPH, Noreen Fertig BS, & Thomas A. Medsger Jr., MD
Summary:
Objective - Epidemiology studies suggest that Systemic Sclerosis is more common, occurs at a younger age and is more severe in African-Americans than Caucasians. However, the scleroderma autoantibody profile is very different between these two ethnic subgroups. This study examines the demographic and disease features, frequency and severity of internal organ system involvement and survival in African-American and Caucasian SSc patients with particular attention to their serum autoantibody profiles.
Methods - Demographic, clinical, autoantibody, organ involvement and survival were studied in consecutive African-American and Caucasian patients seen between 1972 and 2007 as part of the Pittsburgh Scleroderma Database. The Medsger Disease Severity Scale was used to determine severe disease.
Results - African-American patients were more likely to have anti topoisomerase, anti U1RNP and U3 RNP auto-antibodies. Comparing African-American and Caucasians with these antibodies, African-American patients with anti topoisomerase antibody had more frequent and more severe pulmonary fibrosis than Caucasians and an associated decreased survival. Pulmonary fibrosis was also more severe in the U1 RNP patients but was not associated with a difference in survival between African Americans and Caucasians. Anti U3 RNP was associated with more severe gastrointestinal involvement in African-American's compared to Caucasians.
Conclusions - African Americans with systemic sclerosis have more severe disease complications than Caucasians which is associated with both the type of autoantibody and their severity of interstitial lung disease. Hopefully early aggressive intervention in all African Americans with interstitial lung disease will improve outcomes.
Source:
Arthritis & Rheumatism; (2012)