American Indians Have a Higher Risk of Sjögren's Syndrome and More Disease Activity Than European Americans and African Americans

Year: 2019

Type: article

Abstract: OBJECTIVE: To describe the clinical and serologic manifestations of Sjögren's syndrome (SS) in ethnic groups of the US. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Source: Arthritis Care & Research

DOI: 10.1002/acr.24003

Authors: R. Hal Scofield, Rohan Sharma, Nathan Pezant, Jennifer A. Kelly, Lida Radfar, David M. Lewis, C. Erick Kaufman, Sarah Cioli, Judy Harris, Kiely Grundahl, Nelson L. Rhodus, Daniel J. Wallace, Michael H. Weisman, Swamy Venuturupalli, Michael T. Brennan, Kristi A. Koelsch, Christopher J. Lessard, Courtney G. Montgomery, Kathy L. Sivils, Astrid Rasmussen

Institutions: Oklahoma Medical Research Foundation, University of Oklahoma Health Sciences Center, University of Arkansas for Medical Sciences, University of Oklahoma, University of Minnesota, Cedars-Sinai Medical Center, Carolinas Medical Center, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán

Language: English

Topic: Salivary Gland Disorders and Functions

Subfield: Physiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

Funders: U.S. Department of Veterans Affairs, Lupus Research Institute, Oklahoma Medical Research Foundation, National Institutes of Health, National Institute of General Medical Sciences, National Institute of Allergy and Infectious Diseases, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institute of Dental and Craniofacial Research, National Center for Research Resources

OpenAlex ID: W2949917660