Treatment outcomes and their determinants of IgG4-related ophthalmic disease : a territory-wide cohort study
© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ..
BACKGROUND: 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.
STUDY POPULATION: A city-wide, biopsy-proven, Chinese cohort.
METHODS: Retrospective, masked review of medical records, orbital images and histopathology reports.
RESULTS: 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±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).
CONCLUSION: 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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:107 |
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| Enthalten in: |
The British journal of ophthalmology - 107(2023), 12 vom: 22. Nov., Seite 1920-1924 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Lai, Kenneth K H [VerfasserIn] |
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| Links: |
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| Themen: |
Drugs |
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| Anmerkungen: |
Date Completed 24.11.2023 Date Revised 24.11.2023 published: Electronic Citation Status MEDLINE |
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| doi: |
10.1136/bjo-2021-320936 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| 100 | 1 | |a Lai, Kenneth K H |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Treatment outcomes and their determinants of IgG4-related ophthalmic disease |b a territory-wide cohort study |
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| 500 | |a Date Revised 24.11.2023 | ||
| 500 | |a published: Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ. | ||
| 520 | |a BACKGROUND: 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 | ||
| 520 | |a STUDY POPULATION: A city-wide, biopsy-proven, Chinese cohort | ||
| 520 | |a METHODS: Retrospective, masked review of medical records, orbital images and histopathology reports | ||
| 520 | |a RESULTS: 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±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) | ||
| 520 | |a CONCLUSION: 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a drugs | |
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| 650 | 7 | |a Steroids |2 NLM | |
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| 700 | 1 | |a Chan, Karen K W |e verfasserin |4 aut | |
| 700 | 1 | |a Chin, Joyce K Y |e verfasserin |4 aut | |
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| 700 | 1 | |a Io, Ida Y F |e verfasserin |4 aut | |
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