Malignancies in Immunoglobulin G4-related ophthalmic disease. Issue 1 (January 2023)

• Record Type: Journal Article
• Authors: Lai, Kenneth K.H.; Li, Emmy Y.M.; Chan, Regine Y.C.; Wong, Kenneth C.W.; Yu, Jimmy K.S.; Cheuk, W.; Hui, Y.H.; Cheng, Andy C.O.; Chin, Joyce K.Y.; Ip, S. K.; Chan, W. H.; Kwok, Jeremy S.W.; Lam, W. C.; Io, Ida Y. F.; Mak, Theresa S.T.; Li, Kenneth K.W.; Lam, N. M.; Yip, Wilson W. K.; Young, Alvin L.; Chan, Edwin; Ko, Callie K.L.; Ko, Simon T. C.; Yuen, Hunter K.L.; Tham, Clement C.Y.; Pang, Chi Pui; Chong, Kelvin K.L.
• Is Part Of: European journal of ophthalmology. Volume 33:Issue 1(2023)
• Page Start: 171
• Page End: 181
• Publication Date: 2023-01
• Subjects: Immunoglobulin G4 related disease -- immunoglobulin G4 related ophthalmic disease -- ocular adnexal lymphoma -- standardized incidence ratios -- malignancies; Ophthalmology -- Periodicals; Eye -- Diseases -- Periodicals; 617.7005
• DOI: 10.1177/11206721221102274
• Languages: English
• ISSNs: 1120-6721

Abstract

Purpose: Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the patterns of affecting organs have different risks of malignancies. We attempt to determine the association of malignancies with IgG4-related ophthalmic disease (IgG4-ROD). Design: Retrospective cohort study. Methods: Review of medical records, orbital images and histopathology reports in a territory-wide cohort of biopsy proven IgG4-ROD patients from 2005–2019. Findings: Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n = 108), orbital mass (n = 30), infiltrated orbital fat (n = 10), conjunctiva (n = 2) or extraocular muscles (n = 3), 13% (16/122) developed malignancies over 73 ± 48months' follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs = 10.0, 95%CI = 4.5–17.6) while that of extra-orbital malignancies was similar. The SIRs was highest within the first year (SIR = 46.7, 95%CI = 18.5–87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients at IgG4-ROD diagnosis (64.9 ± 7.1, 68.3 ± 8.5 versus 55.2 ± 15.0 years, P < 0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%) were associated with OAL (all P < 0.05). Pre-treatment serum IgG4 level or extra-orbital IgG4-RD involvement was similar among patients with or without malignancies. Conclusion: In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.