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Review
. 2024 Oct;32(8):1852-1858.
doi: 10.1080/09273948.2023.2280709. Epub 2023 Dec 6.

Systemic Involvement in Immunoglobulin G4-Related Ophthalmic Disease

Kenneth K H Lai  1   2 , Fatema Mohamed Ali Abdulla Aljufairi  2   3 , Jake Uy Sebastian  2   4 , Carson C Y Yip  2 , Yingying Wei  5 , Ruofan Jia  5 , Wah Cheuk  6 , Andy C O Cheng  7 , Joyce K Y Chin  8 , Chung Yin Chu  9 , Chi Ho Kwong  10 , Nelson K F Yip  11 , Kenneth K W Li  11 , W H Chan  12 , Wilson W K Yip  8 , Alvin L Young  8 , Edwin Chan  1 , Callie K L Ko  1 , Carmen K M Chan  2   13 , Hunter K L Yuen  2   13 , Li Jia Chen  2   8 , Clement C Y Tham  2   8 , Chi Pui Pang  2 , Kelvin K L Chong  2   8   13
Affiliations
Review

Systemic Involvement in Immunoglobulin G4-Related Ophthalmic Disease

Kenneth K H Lai et al. Ocul Immunol Inflamm. 2024 Oct.

Abstract

Background: Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) poses clinical challenges due to its heterogeneous ocular and systemic manifestations. We aim to report the systemic involvement and the clinical, serological and radiological associations of a cohort of Chinese patients.

Methods: A territory-wide, biopsy-proven, Chinese cohort. A retrospective, masked chart review of medical records, orbital images, and histopathology reports.

Results: A total of 122 (65 male) patients with a follow-up of 81 ± 49 (24 to 84) months were reviewed. Ninety (74%) patients presented bilaterally. Subacute upper eyelid swelling was the commonest presentation (82/122, 67%). During follow-up, 91/122 patients (75%) underwent extra-orbital imaging including computer tomography (692 films), ultrasonography (182 films), magnetic resonance imaging (76 films) and whole body FDG-PET scan (33 films). Eighty-six (95%) of these 91 patients had extra-orbital involvement radiologically (2.7 ± 1.6 regions, range: 0 to 9). Lymph node was the most prevalent (N = 60,66%), followed by salivary gland (N = 51,56%), lung (N = 49,54%), kidney (N = 22, 24%), hepatobiliary tree (N = 18, 20%) and pancreas (N = 17, 19%). Other organs include thyroid, aorta, meninges/brain and skin. Twenty-eight (23%) patients had allergic diseases (19 asthma, 16 allergic rhinitis, and 6 eczemas). Fifty-seven (48%) patients had paranasal sinusitis. Serum eosinophilia was associated with a higher number (3.24 versus 2.52, P = 0.0304) of organ involvement. Patients with deep organ involvement was associated with a higher age of IgG4-ROD onset (70 ± 12 versus 56 ± 13, P < 0.0001).

Conclusions: 95% of the patients who underwent systemic imaging in our cohort had systemic organ involvement. An early physicians' assessment and radiological imaging are recommended after the diagnosis of IgG4-ROD.

Keywords: Immunoglobulin G4; immunoglobulin G4-related ophthalmic disease; systemic involvement.

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