Clinical Characteristics and Burden of IgG4-Related Disease in the United Arab Emirates

Abstract

Background

There is a scarcity of literature detailing the demographic and clinical data concerning immunoglobulin G4–related disease (IgG4-RD) within the population of the UAE.

Objective

This retrospective follow-up study aimed to investigate the demographic and clinical characteristics of IgG4-RD in an Arab cohort receiving treatment at Cleveland Clinic Abu Dhabi.

Patients and Methods

Data were collected from 28 Arab patients diagnosed with IgG4-RD at Cleveland Clinic Abu Dhabi between April 1, 2015 and May 31, 2023. The study employed rigorous inclusion and exclusion criteria, utilizing the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2019 criteria for classification. Comprehensive analysis of electronic health records included demographics, clinical history, laboratory results, and treatment specifics. The study received ethical approval.

Results

The median age of the patients at the time of initial symptoms was 42 ± 3 years (median ± standard deviation) and that at the time of diagnosis was 47 ± 4.6 years, with a 5-year lag period between initial clinical presentation and establishing a diagnosis. The most common symptoms were constitutional symptoms (71%) followed by low back pain experienced by 54% of patients due to mass effect. In terms of organ involvement, 89% of patients exhibited a single organ being affected. The kidney was the most frequently involved organ (46%). Treatment involved glucocorticoids, often combined with methotrexate and azathioprine, with rituximab.

Conclusion

The study highlights unique features in the Arab population, including a younger median age and the absence of pancreatic involvement. The findings emphasize the need for a national registry to understand disease prevalence, raise awareness, and guide health care strategies for IgG4-RD.

Keywords

IgG4 syndrome - IgG4-RD - IgG4-related autoimmune disease - IgG4-related sclerosing disease

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