TY - JOUR
T1 - Anti-fibrillarin antibody in African American patients with systemic sclerosis
T2 - Immunogenetics, clinical features, and survival analysis
AU - Sharif, Roozbeh
AU - Fritzler, Marvin J.
AU - Mayes, Maureen D.
AU - Gonzalez, Emilio B.
AU - McNearney, Terry A.
AU - Draeger, Hilda
AU - Baron, Murray
AU - Furst, Daniel E.
AU - Khanna, Dinesh K.
AU - Del Junco, Deborah J.
AU - Molitor, Jerry A.
AU - Schiopu, Elena
AU - Phillips, Kristine
AU - Seibold, James R.
AU - Silver, Richard M.
AU - Simms, Robert W.
AU - Perry, Marilyn
AU - Rojo, Carlos
AU - Charles, Julio
AU - Zhou, Xiaodong
AU - Agarwal, Sandeep K.
AU - Reveille, John D.
AU - Assassi, Shervin
AU - Arnett, Frank C.
AU - Pope, Janet E.
AU - Markland, Janet
AU - Robinson, David
AU - Jones, Niall
AU - Khalidi, Nader
AU - Docherty, Peter
AU - Abu-Hakima, Maysan
AU - LeClercq, Sharon
AU - Sutton, Evelyn
AU - Smith, Douglas
AU - Mathieu, Jean Pierre
AU - Masetto, Alejandra
AU - Kaminska, Elzbieta
AU - Ligier, Sophie
PY - 2011/8
Y1 - 2011/8
N2 - 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. The Journal of Rheumatology
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. The Journal of Rheumatology
KW - Anti-U3-RNP
KW - Digital ulcer
KW - Genisos
KW - HLA-DRB1
KW - Scleroderma
KW - Scleroderma family registry
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U2 - 10.3899/jrheum.110071
DO - 10.3899/jrheum.110071
M3 - Article
C2 - 21572159
AN - SCOPUS:79961109536
SN - 0315-162X
VL - 38
SP - 1622
EP - 1630
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 8
ER -
