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

Authors' Contributions

R.N. contributed to the conception and design of the study, drafting of the article, and final approval of the manuscript. H.A., M.A., and R.E. contributed to data analysis and interpretation of data, and final approval of the manuscript. S.A.Q. contributed to data analysis and interpretation of data, drafting of the article, and final approval of the manuscript. E.M., F.S., A.M., A.A., M.C., S.H., M.M., M.A., M.E., and M.G. contributed to critical revision and final approval of the manuscript. A.A.M. contributed to critical revision, drafting of the article, and final approval of the manuscript.

Compliance with Ethical Principles

The study was approved by the Institutional Research Board (IRB) under study number A-2020-070, with all participants providing consent.

Publikationsverlauf

Artikel online veröffentlicht:
14. Februar 2025

• References

• 1 Deshpande V, Zen Y, Chan JK. et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012; 25 (09) 1181-1192
• 2 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366 (06) 539-551
• 3 Wang L, Zhang P, Zhang X. et al. Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients. Rheumatology 2019; 58 (05) 820-830
• 4 Martínez-Valle F, Fernández-Codina A, Pinal-Fernández I, Orozco-Gálvez O, Vilardell-Tarrés M. IgG4-related disease: evidence from six recent cohorts. Autoimmun Rev 2017; 16 (02) 168-172
• 5 Wallace ZS, Naden RP, Chari S. et al; Members of the ACR/EULAR IgG4-RD Classification Criteria Working Group. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis 2020; 79 (01) 77-87
• 6 Wallace ZS, Deshpande V, Mattoo H. et al. IgG4-related disease: clinical and laboratory features in 125 patients. Arthritis Rheum 2015; 67 (09) 2466-2475
• 7 Karadağ Ö, Erden A, Ayhan EA. et al. The clinical features and outcomes of Turkish patients with IgG4-related disease: a single-center experience. Turk J Med Sci 2017; 47 (05) 1307-1314
• 8 Namas R, Elarabi M, Hegazin S. et al. Clinical characteristics of IgG4-related disease in the United Arab Emirates: a retrospective single-center study. Ibnosina J Med Biomed Sci 2021; 13: 60-66
• 9 Umehara H, Okazaki K, Nakamura T. et al. Current approach to the diagnosis of IgG4-related disease: combination of comprehensive diagnostic and organ-specific criteria. Mod Rheumatol 2017; 27 (03) 381-391
• 10 Abdulnasser A, Hussein M, Ahmed I. et al. Clinical characteristics of IgG4-related disease in Saudi Arabia: a multicenter retrospective cohort. Saudi J Med Sci. 2020; 8: 24-29
• 11 Quero M, Draibe J, Solanich X. et al. Clinical features and outcomes in a cohort of patients with immunoglobulin G4-related disease at a university hospital in Spain. Clin Kidney J 2019; 12 (06) 829-835
• 12 Inoue D, Yoshida K, Yoneda N. et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore) 2015; 94 (15) e680
• 13 Lee CM, Alalwani M, Prayson RA, Gota CE. Retrospective single-centre analysis of IgG4-related disease patient population and treatment outcomes between 2007 and 2017. Rheumatol Adv Pract 2019; 3 (01) rkz014
• 14 Fernández-Codina A, Martínez-Valle F, Pinilla B. et al. IgG4-related disease: results from a multicenter Spanish registry. Medicine (Baltimore) 2015; 94 (32) e1275
• 15 Khosroshahi A, Wallace ZS, Crowe JL. et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol 2015; 67 (07) 1688-1699
• 16 Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet 2015; 385 (9976): 1460-1471
• 17 Carruthers MN, Topazian MD, Khosroshahi A. et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis 2015; 74 (06) 1171-1177
• 18 Ebbo M, Daniel L, Pavic M. et al. IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine (Baltimore) 2012; 91 (01) 49-56
• 19 Campochiaro C, Ramirez GA, Bozzolo EP. et al. IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients. Scand J Rheumatol 2016; 45 (02) 135-145
• 20 Lin W, Lu S, Chen H. et al. Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients. Rheumatology (Oxford) 2015; 54 (11) 1982-1990
• 21 Evans RDR, Cargill T, Goodchild G. et al. Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort. Kidney Int Rep 2018; 4 (01) 48-58