RT Journal Article SR Electronic T1 Anti-Fibrillarin Antibody in African American Patients with Systemic Sclerosis: Immunogenetics, Clinical Features, and Survival Analysis JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP 1622 OP 1630 DO 10.3899/jrheum.110071 VO 38 IS 8 A1 ROOZBEH SHARIF A1 MARVIN J. FRITZLER A1 MAUREEN D. MAYES A1 EMILIO B. GONZALEZ A1 TERRY A. McNEARNEY A1 HILDA DRAEGER A1 MURRAY BARON A1 the Canadian Scleroderma Research Group A1 DANIEL E. FURST A1 DINESH K. KHANNA A1 DEBORAH J. DEL JUNCO A1 JERRY A. MOLITOR A1 ELENA SCHIOPU A1 KRISTINE PHILLIPS A1 JAMES R. SEIBOLD A1 RICHARD M. SILVER A1 ROBERT W. SIMMS A1 GENISOS Study Group A1 MARILYN PERRY A1 CARLOS ROJO A1 JULIO CHARLES A1 XIAODONG ZHOU A1 SANDEEP K. AGARWAL A1 JOHN D. REVEILLE A1 SHERVIN ASSASSI A1 FRANK C. ARNETT YR 2011 UL http://www.jrheum.org/content/38/8/1622.abstract AB Objective. Anti-U3-RNP, or anti-fibrillarin antibodies (AFA), are detected more frequently among African American (AA) patients with systemic sclerosis (SSc) compared to other ethnic groups and are associated with distinct clinical features. We examined the immunogenetic, clinical, and survival correlates of AFA in a large group of AA patients with SSc. Methods. Overall, 278 AA patients with SSc and 328 unaffected AA controls were enrolled from 3 North American cohorts. Clinical features, autoantibody profile, and HLA class II genotyping were determined. To compare clinical manifestations, relevant clinical features were adjusted for disease duration. Cox proportional hazards regression was used to determine the effect of AFA on survival. Results. Fifty (18.5%) AA patients had AFA. After Bonferroni correction, HLA-DRB1*08:04 was associated with AFA, compared to unaffected AA controls (OR 11.5, p < 0.0001) and AFA-negative SSc patients (OR 5.2, p = 0.0002). AFA-positive AA patients had younger age of disease onset, higher frequency of digital ulcers, diarrhea, pericarditis, higher Medsger perivascular and lower Medsger lung severity indices (p = 0.004, p = 0.014, p = 0.019, p = 0.092, p = 0.006, and p = 0.016, respectively). After adjustment for age at enrollment, AFA-positive patients did not have different survival compared to patients without AFA (p = 0.493). Conclusion. Our findings demonstrate strong association between AFA and HLA-DRB1*08:04 allele in AA patients with SSc. AA SSc patients with AFA had younger age of onset, higher frequency of digital ulcers, pericarditis and severe lower gastrointestinal involvement, but less severe lung involvement compared to AA patients without AFA. Presence of AFA did not change survival.