Anti-Fibrillarin Antibody in African American Patients with Systemic Sclerosis: Immunogenetics, Clinical Features, and Survival Analysis
Anti-Fibrillarin Antibody in African American Patients with Systemic Sclerosis: Immunogenetics, Clinical Features, and Survival Analysis
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DOI：
10.3899/jrheum.110071
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发表时间：
2011-08-01
影响因子：
3.9
通讯作者：
Arnett, Frank C.
中科院分区：
文献类型：
--
作者：
Sharif, Roozbeh;Fritzler, Marvin J.;Arnett, Frank C.
Objective.抗U3-RNP或抗原纤维蛋白抗体（AFA）在患有系统性硬化症（SSc）的非裔美国人（AA）患者中的检出率高于其他种族，并且与不同的临床特征相关。我们研究了一个大组的AA患者与SSc的免疫遗传学，临床和生存相关的AFA。总体而言，从3个北美队列入组了278例SSc AA患者和328例未受影响的AA对照。确定临床特征、自身抗体谱和HLA II类基因分型。为了比较临床表现，调整了疾病持续时间的相关临床特征。采用考克斯比例风险回归分析AFA对生存率的影响。50例（18.5%）AA患者有AFA。Bonferroni校正后，HLA-DRB 1 *08：04与AFA相关，与未受影响的AA对照（OR 11.5，p < 0.0001）和AFA阴性SSc患者（OR 5.2，p = 0.0002）相比。AFA阳性AA患者的发病年龄更小，手指溃疡、腹泻、心包炎的发生率更高，Medsger血管周围指数更高，Medsger肺严重程度指数更低（分别为p = 0.004、p = 0.014、p = 0.019、p = 0.092、p = 0.006和p = 0.016）。校正入组时的年龄后，AFA阳性患者与无AFA患者的生存率无差异（p = 0.493）。我们的研究结果表明，AA患者SSc中AFA和HLA-DRB 1*08：04等位基因之间存在强相关性。与无AFA的AA患者相比，AFA SSc患者的发病年龄更小，手指溃疡、心包炎和严重下消化道受累的频率更高，但肺部受累的严重程度较低。AFA的存在并没有改变生存率。（2011年5月15日首次发布; J Rheumol 2011;38：1622-30; doi：10.3899/jrheum.110071）
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-DRB 1*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. (First Release May 15 2011; J Rheumatol 2011;38:1622-30; doi :10.3899/jrheum.110071)