Clinical characteristics of igG4-related disease in the United Arab Emirates: A retrospective single-center study

Rajaie Namas; Mohamed Elarabi; Safa Hegazin; Abhay Joshi; Fulvio Salvo; Imad Jassim; Muriel Ghosn; Nizar Attallah; Priti Joshi; Esat Memisoglu; Waleed Hassen; Jamal Al-Saleh; Mohamed Abuzakouk

doi:10.4103/ijmbs.ijmbs_13_21

Ibnosina Journal of Medicine and Biomedical Sciences, Table of Contents

CC BY-NC-ND 4.0 · Ibnosina Journal of Medicine and Biomedical Sciences 2021; 13(02): 60-66
DOI: 10.4103/ijmbs.ijmbs_13_21

Original Article

Clinical characteristics of igG4-related disease in the United Arab Emirates: A retrospective single-center study

Rajaie Namas

¹Department of Internal Medicine, Division of Rheumatology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Mohamed Elarabi

¹Department of Internal Medicine, Division of Rheumatology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Safa Hegazin

²Department of Internal Medicine, Division of Hospital Medicine, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Abhay Joshi

¹Department of Internal Medicine, Division of Rheumatology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Fulvio Salvo

³Department of Medicine, Division of Allergy and Immunology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Imad Jassim

⁴Department of Medicine, Division of Rheumatology, Tawam Hospital, Al-Ain

,

Muriel Ghosn

⁵Department of Internal Medicine, Division of Nephrology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Nizar Attallah

⁵Department of Internal Medicine, Division of Nephrology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Priti Joshi

⁶Department of Medicine, Division of Pathology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Esat Memisoglu

⁷Department of Radiology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Waleed Hassen

⁸Department of Medicine, Division of Urology, Cleveland Clinic Abu Dhabi, Abu Dhabi

,

Jamal Al-Saleh

⁹Department of Internal Medicine, Division of Rheumatology, Dubai Hospital, Dubai

,

Mohamed Abuzakouk

³Department of Medicine, Division of Allergy and Immunology, Cleveland Clinic Abu Dhabi, Abu Dhabi

› Author Affiliations

Abstract

Background: Demographic and clinical data of IgG4-related disease affecting solely the native population of the UAE, known as Emiratis, does not exist in the literature. Aim: To explore the demographic and clinical characteristics of IgG4-related disease in a well-defined population of Emirati patients attending Cleveland Clinic Abu Dhabi, a large tertiary center in the Middle East. Patients and Methods: The data presented is part of a retrospective cohort study, in which 15 Emirati patients with IgG4-related disease (IgG4-RD) were evaluated over 5 years from April 2015 to September 2020 at the rheumatology outpatient clinic at Cleveland Clinic Abu Dhabi. The demographic and clinical data were recorded. Descriptive statistics of the variables were applied. Results: Fifteen Emirati patients with an established diagnosis of IgG4-RD were assessed. There was a male predominance (53%) with a median age at the time of diagnosis of 47 ± 11.2 years. A 6-year lag period was noted from the initial presentation until a diagnosis of IgG4-RD was established. The most frequent comorbidities observed were hematological conditions (63%), hypertension (47%), diabetes mellitus (40%), and gastroesophageal reflux disease (40%). An elevated serum IgG4 was observed in the majority of patients at the initial presentation. Rheumatoid factor was detected in 13% and low titer of immunofluorescence antinuclear antibody in 7%. 86% of patients had a tissue biopsy with marked lymphocytic and plasmacytic infiltration being the most reported finding in 86%. Methotrexate, azathioprine, and rituximab were the most frequently prescribed disease-modifying agents. Conclusion: We report the first comprehensive analysis on a small cohort of Emirati patients with IgG4-RD. We describe disease features unique to UAE patients and demonstrate that IgG4-RD has a significant disease burden. Our results underscore the need for the IgG4-RD UAE-wide national registry to improve the quality of care of these patients.

Key-words:

IgG4 - IgG4-RD - native-populations - rheumatology - UAE

Full Text

References

References
1 Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-92.
2 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539-51.
3 Quero M, Draibe J, Solanich X, Rama I, Gomà M, Martínez-Valenzuela L, 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;12:829-35.
4 Wang L, Zhang P, Zhang X, Lin W, Tang H, Li J, et al. Sex disparities in clinical characteristics and prognosis of immunoglobulin G4–related disease: A prospective study of 403 patients. Rheumatology 2019;58:820-30.
5 Wallace ZS, Naden RP, Chari S, Choi HK, Della-Torre E, Dicaire JF, et al. The 2019 American College of Rheumatology/European League against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis 2020;79:77-87.
6 Umehara H, Okazaki K, Nakamura T, Satoh-Nakamura T, Nakajima A, Kawano M, et al. Current approach to the diagnosis of IgG4-related disease – Combination of comprehensive diagnostic and organ-specific criteria. Mod Rheumatol 2017;27:381-91.
7 Martínez-Valle F, Fernández-Codina A, Pinal-Fernández I, Orozco-Gálvez O, Vilardell-Tarrés O. IgG4-related disease: Evidence from six recent cohorts. Autoimmun Rev 2017;16:168-72.
8 Wallace ZS, Deshpande V, Mattoo H, Mahajan VS, Kulikova M, Pillai S, et al. IgG4-related disease: Clinical and laboratory features in 125 patients. Arthritis Rheum 2015;67:2466-75.
9 Karadağ Ö, Erden A, Ayhan EA, Bölek EÇ, Kalyoncu U, Armağan B, et al. The clinical features and outcomes of Turkish patients with IgG4-related disease: A single-center experience Turk J Med Sci 2017;47:1307-14.
10 Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K, et al. IgG4-related disease: Dataset of 235 consecutive patients. Medicine (Baltimore) 2015;94:e680.
11 Ebbo M, Daniel L, Pavic M, Sève P, Hamidou M, Andres E, et al. IgG4-related systemic disease: Features and treatment response in a French cohort: Results of a multicenter registry. Medicine (Baltimore) 2012;91:49-56.
12 Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, et al. Rituximab for IgG4-related disease: A prospective, open-label trial. Ann Rheum Dis 2015;74:1171-7.
13 Campochiaro C, Ramirez GA, Bozzolo EP, Lanzillotta M, Berti A, Baldissera E, et al. IgG4-related disease in Italy: Clinical features and outcomes of a large cohort of patients. Scand J Rheumatol 2016;45:135-45.
14 Lin W, Lu S, Chen H, Wu Q, Fei Y, Li M, et al. Clinical characteristics of immunoglobulin G4-related disease: A prospective study of 118 Chinese patients. Rheumatology (Oxford) 2015;54:1982-90.
15 Fernández-Codina A, Martínez-Valle F, Pinilla B, López C, DeTorres I, Solans-Laqué R, et al. IgG4-related disease: Results from a multicenter Spanish registry. Medicine (Baltimore) 2015;94:e1275.