Journal Article

Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study

Li, Emmy Y M, Io, Ida Y F, Chu, Winnie C W, Yip, Wilson W K, Yip, Nelson K F, Chan, Regine Y C, Yuen, Hunter K L, Pang, Chi-Pui, Lam, Nai Man, Ko, Callie K L, Cheng, Andy C O, Chan, Wai Ho, Ko, Simon T C, Kwok, Jeremy S W, Young, Alvin L, Li, Kenneth K W, Chan, Karen K W, Lai, Kenneth K H, Chin, Joyce K Y, Chong, Kelvin K L, Chan, Edwin, Tham, Clement C Y

2023

Overview

BackgroundOral corticosteroid remains the first-line treatment of IgG4-related ophthalmic disease, but steroid-dependence is common and serious. Factors associated with steroid dependence and relapse have to be further explored.Study populationA city-wide, biopsy-proven, Chinese cohort.MethodsRetrospective, masked review of medical records, orbital images and histopathology reports.ResultsThere were 101 patients with at least 24-month follow-up. Up to 82% (82/101) received oral corticosteroid as first-line treatments, and 7 of them received also concomitant steroid-sparing agents (SSA)/biological agents as primary treatment. There was 61% (50/82) of patients required long-term corticosteroid (alone=23, with SSA=27) after 1.9±0.7 (range 1–5) relapses. When compared with the 21% (17/82) of patients who tapered corticosteroid successfully for 24 months, steroid dependence was associated with elevated baseline serum IgG4 level (94% vs 65%, p<0.01) and Mikulicz syndrome (46% vs 18%, p<0.05). Up to 13% (11/82) of patients tolerated residual disease after tapering off corticosteroid. There was 17% (17/101) of patients did not require any medications after biopsies. They were more likely to have debulking surgeries (71% vs 40%, p<0.05), discrete orbital lesions (65% vs 26%, p<0.05), normal baseline serum IgG4 level (24% vs 6%, p<0.05) and no Mikulicz syndrome (94% vs 61%, p<0.05).ConclusionIn this cohort, 60% of patients required long-term maintenance oral corticosteroid. Elevated pretreatment serum IgG4 level and Mikulicz syndrome were associated with steroid dependence. Debulking surgery is an alternative for a subgroup of patients with discrete orbital lesions, normal baseline IgG4 level and no Mikulicz syndrome.

• Publisher: BMJ Publishing Group Ltd,BMJ Publishing Group LTD
• Subject: Asthma / Biopsy / Chemotherapy / Clinical outcomes / Clinical science / Cohort analysis / Data analysis / Drug dosages / drugs / Eye diseases / Eye surgery / Hay fever / immunology / inflammation / Inflammatory diseases / Lymphoma / Ophthalmology / orbit / Patients / Remission (Medicine) / Rhinitis / Steroids / Surgery / Treatment refusal