Download PDF
CC BY 4.0 · Ibnosina Journal of Medicine and Biomedical Sciences 2025; 17(01): 018-024
DOI: 10.1055/s-0045-1802561
Original Article

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

Rajaie Namas 
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Hamdan Alawadhi
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Mahra AlMheiri
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Rema Eljabour
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Sarah Al Qassimi
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Esat Memisoglu
2   Department of Radiology, Imaging Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Fulvio Salvo
3   Division of Allergy and Immunology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Asia Mubashir
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Amer AlKhatib
4   Division of Gastroenterology and Hepatology, Digestive Diseases Institute, Cleveland Clinic Abu Dhabi, United Arab Emirates
,
Maguy Chiha
5   Division of Endocrinology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Safa Hegazin
6   Division of General Surgery, Digestive Diseases Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Mohamad Masri
7   Division of Hematology and Medical Oncology, Oncology Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Mohamed Abuzakouk
3   Division of Allergy and Immunology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Mohamed Elarabi
1   Division of Rheumatology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
,
Ahlam Almarzooqi
8   Department of Rheumatology, Al Qassimi Hospital, Emirates Health Services, Sharjah, United Arab Emirates
,
Muriel Ghosn
9   Division of Nephrology, Department of Internal Medicine, Medical Subspecialties Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
› Author Affiliations
Funding and Sponsorship None.
› Further Information
Also available at
  Permissions and Reprints

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.




Publication History

Article published online:
14 February 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • 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
    CrossrefPubMedSearch in Google Scholar
  • 2 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366 (06) 539-551
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    Thieme ConnectSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    Search in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 12 Inoue D, Yoshida K, Yoneda N. et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore) 2015; 94 (15) e680
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 16 Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet 2015; 385 (9976): 1460-1471
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar