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

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.MethodsRetros...

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Published in:British journal of ophthalmology Vol. 107; no. 12; pp. 1920 - 1924
Main Authors: Lai, Kenneth K H, Li, Emmy Y M, Chan, Regine Y C, Chu, Winnie C W, Cheng, Andy C O, Chan, Karen K W, Chin, Joyce K Y, Kwok, Jeremy S W, Io, Ida Y F, Yip, Nelson K F, Li, Kenneth K W, Chan, Wai Ho, Lam, Nai Man, 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
Format: Journal Article
Language:English
Published: BMA House, Tavistock Square, London, WC1H 9JR BMJ Publishing Group Ltd 01.12.2023
BMJ Publishing Group LTD
Subjects:
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
China
Hong Kong China
ISSN:0007-1161, 1468-2079, 1468-2079
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Summary: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.
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ISSN:0007-1161
1468-2079
1468-2079
DOI:10.1136/bjo-2021-320936