{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T01:48:47Z","timestamp":1790300927228,"version":"4.1.0"},"reference-count":34,"publisher":"BMJ","issue":"12","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Br J Ophthalmol"],"accepted":{"date-parts":[[2022,10,5]]},"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Oral 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.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Study population<\/jats:title>\n<jats:p>A city-wide, biopsy-proven, Chinese cohort.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>Retrospective, masked review of medical records, orbital images and histopathology reports.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n    <jats:p>There 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\u00b10.7 (range 1\u20135) 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&lt;0.01) and Mikulicz syndrome (46% vs 18%, p&lt;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&lt;0.05), discrete orbital lesions (65% vs 26%, p&lt;0.05), normal baseline serum IgG4 level (24% vs 6%, p&lt;0.05) and no Mikulicz syndrome (94% vs 61%, p&lt;0.05).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>In 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.<\/jats:p>\n<\/jats:sec>","DOI":"10.1136\/bjo-2021-320936","type":"journal-article","created":{"date-parts":[[2022,10,26]],"date-time":"2022-10-26T17:20:33Z","timestamp":1666804833000},"page":"1920-1924","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":10,"title":["Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study"],"prefix":"10.1136","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9579-2060","authenticated-orcid":false,"given":"Kenneth K H","family":"Lai","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmy Y M","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Regine Y 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