Oklahoma Medical Research Foundation, University of Oklahoma Health Sciences Center, and Department of Veterans Affairs Medical Center, Oklahoma City.
University of Arkansas for Medical Sciences, Little Rock.
Arthritis Care Res (Hoboken). 2020 Aug;72(8):1049-1056. doi: 10.1002/acr.24003. Epub 2020 Jul 5.
To describe the clinical and serologic manifestations of Sjögren's syndrome (SS) in ethnic groups of the US.
This was a cross-sectional study of 648 patients with primary SS: 20 African American (AA), 164 American Indian (AI), 426 European American (EA), and 38 patients of other races evaluated in a multidisciplinary Sjögren's International Collaborative Clinical Alliance research clinic.
AA subjects comprised 3.1% of the SS cohort, much lower than the percentage of AA in the state of Oklahoma (P = 3.01 × E - 05), the US (P = 2.24E - 13), or a systemic lupus erythematosus (SLE) cohort at the same institution (P = 4.26 × 10E - 27). In contrast, the percentage of AI in the SS cohort (25.3%) was much higher than expected (P = 3.17E - 09 versus SLE cohort, P = 6.36 - 26 versus Oklahoma, and P = 8.14E - 96 versus US population). The SS classification criteria were similar between AA and EA, but subjects of AI ancestry had lower rates of abnormal tear and salivary flow, as well as anti-Ro/SSA and anti-La/SSB antibodies. Paradoxically, AI had higher levels of disease activity (mean ± SD European League Against Rheumatism Sjögren's Syndrome Disease Activity Index score 3.77 ± 4.78) in comparison to EA (2.90 ± 4.12; P = 0.011) and more extraglandular manifestations affecting mainly the articular and glandular domains. Meanwhile, AA patients were characterized by higher rates of hypergammaglobulinemia (odds ratio [OR] 1.39 [95% confidence interval (95% CI) 1.39-8.65]; P = 0.01), elevated erythrocyte sedimentation rate (ESR) (OR 3.95 [95% CI 1.46-9.95]; P = 0.009), and parotid enlargement (OR 4.40 [95% CI 1.49-13.07]; P = 0.02).
AI are affected at high rates with SS but present with few classical features, potentially preventing timely diagnosis. In contrast to SLE, SS is infrequent and not more severe among AA, but the triad of hypergammaglobulinemia, increased ESR, and parotid enlargement warrants extra vigilance for lymphomagenesis.
描述美国不同种族人群干燥综合征（SS）的临床和血清学表现。
这是一项对 648 例原发性 SS 患者的横断面研究：20 例非裔美国人（AA）、164 例美洲印第安人（AI）、426 例欧洲裔美国人（EA）和 38 例其他种族患者在一个多学科干燥综合征国际协作临床联盟研究诊所接受评估。
AA 患者占 SS 队列的 3.1%，远低于俄克拉荷马州（P = 3.01E-05）、美国（P = 2.24E-13）或同一机构系统性红斑狼疮（SLE）队列中非裔美国人的比例（P = 4.26E-27）。相比之下，SS 队列中 AI 的比例（25.3%）远高于预期（P = 3.17E-09 与 SLE 队列，P = 6.36-26 与俄克拉荷马州，P = 8.14E-96 与美国人口）。AA 和 EA 患者的 SS 分类标准相似，但 AI 患者的异常泪液和唾液流量以及抗 Ro/SSA 和抗 La/SSB 抗体的发生率较低。矛盾的是，与 EA 相比（2.90 ± 4.12；P = 0.011），AI 的疾病活动度更高（平均 ± 标准差欧洲抗风湿病联盟干燥综合征疾病活动指数评分 3.77 ± 4.78），并且更多的关节外表现主要影响关节和腺体。与此同时，AA 患者的高丙种球蛋白血症（比值比 [OR] 1.39 [95%置信区间（95%CI）1.39-8.65]；P = 0.01）、红细胞沉降率（ESR）升高（OR 3.95 [95%CI 1.46-9.95]；P = 0.009）和腮腺肿大（OR 4.40 [95%CI 1.49-13.07]；P = 0.02）的发生率更高。
AI 患 SS 的比例很高，但表现出的典型特征很少，可能会导致诊断不及时。与 SLE 不同，AA 中 SS 并不常见且不更严重，但高丙种球蛋白血症、ESR 升高和腮腺肿大三联征提示需要加强对淋巴瘤发生的警惕。
