PT  - JOURNAL ARTICLE
AU  - ROOZBEH SHARIF
AU  - MARVIN J. FRITZLER
AU  - MAUREEN D. MAYES
AU  - EMILIO B. GONZALEZ
AU  - TERRY A. McNEARNEY
AU  - HILDA DRAEGER
AU  - MURRAY BARON
AU  - the Canadian Scleroderma Research Group
AU  - DANIEL E. FURST
AU  - DINESH K. KHANNA
AU  - DEBORAH J. DEL JUNCO
AU  - JERRY A. MOLITOR
AU  - ELENA SCHIOPU
AU  - KRISTINE PHILLIPS
AU  - JAMES R. SEIBOLD
AU  - RICHARD M. SILVER
AU  - ROBERT W. SIMMS
AU  - GENISOS Study Group
AU  - MARILYN PERRY
AU  - CARLOS ROJO
AU  - JULIO CHARLES
AU  - XIAODONG ZHOU
AU  - SANDEEP K. AGARWAL
AU  - JOHN D. REVEILLE
AU  - SHERVIN ASSASSI
AU  - FRANK C. ARNETT
TI  - Anti-Fibrillarin Antibody in African American Patients with Systemic Sclerosis: Immunogenetics, Clinical Features, and Survival Analysis
AID  - 10.3899/jrheum.110071
DP  - 2011 Aug 01
TA  - The Journal of Rheumatology
PG  - 1622--1630
VI  - 38
IP  - 8
4099  - http://www.jrheum.org/content/38/8/1622.short
4100  - http://www.jrheum.org/content/38/8/1622.full
SO  - J Rheumatol2011 Aug 01; 38
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.