Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance

(1) Background: To analyze the association between long-term changes in serum IgG4 levels and the clinical course of patients with IgG4-related ophthalmic disease (IgG4-ROD). (2) Methods: Retrospective analysis of 25 patients with IgG4-ROD. (3) Results: Mean age at diagnosis was 60.68 years. Fifty-s...

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Main Authors: Wei-Yi Chou, Ching-Yao Tsai, Chieh-Chih Tsai
Format: Article
Language:English
Published: MDPI AG 2022-11-01
Series:Journal of Personalized Medicine
Subjects:
Online Access:https://www.mdpi.com/2075-4426/12/12/1963
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author Wei-Yi Chou
Ching-Yao Tsai
Chieh-Chih Tsai
author_facet Wei-Yi Chou
Ching-Yao Tsai
Chieh-Chih Tsai
author_sort Wei-Yi Chou
collection DOAJ
description (1) Background: To analyze the association between long-term changes in serum IgG4 levels and the clinical course of patients with IgG4-related ophthalmic disease (IgG4-ROD). (2) Methods: Retrospective analysis of 25 patients with IgG4-ROD. (3) Results: Mean age at diagnosis was 60.68 years. Fifty-six percent of patients had bilateral ocular involvement and 32% had systemic associations. The ocular structures involved were the lacrimal gland (76%), orbital soft tissue (36%), extraocular muscle (20%) and infraorbital nerve (20%). According to last follow-up, 9 (36%) patients had normalized IgG4 levels, and 16 (64%) patients had elevated IgG4 levels. Patients with normalized IgG4 levels had better response to initial steroid treatment and attained a significantly lower IgG4 level after treatment (<i>p</i> = 0.002). The highest IgG4 levels were at baseline and disease recurrence, and lowest after initial treatment. At final follow-up, IgG4 levels differed in patients with remission (mean 326.25 mg/dL) and stable disease (mean 699.55 mg/dL). Subgroup analysis was performed in patients with remission, categorized according to whether IgG4 levels were normalized (9 patients) or elevated (10 patients) on last follow up. The elevated group had a higher percentage of bilateral disease, lacrimal gland involvement and recurrence. (4) Conclusions: IgG4-ROD patients with a greater response to initial steroid therapy were more inclined to have normalized IgG4 levels in the long term. Some patients remained in remission despite persistently elevated IgG4 levels, and had regular follow-up without treatment.
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spelling doaj.art-4967392b3f9f473190134bf8bc0dc15e2023-11-24T16:01:16ZengMDPI AGJournal of Personalized Medicine2075-44262022-11-011212196310.3390/jpm12121963Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical RelevanceWei-Yi Chou0Ching-Yao Tsai1Chieh-Chih Tsai2Department of Ophthalmology, Zhongxing Branch, Taipei City Hospital, Taipei 103212, TaiwanDepartment of Ophthalmology, Zhongxing Branch, Taipei City Hospital, Taipei 103212, TaiwanDepartment of Ophthalmology, Taipei Veterans General Hospital, Taipei 11217, Taiwan(1) Background: To analyze the association between long-term changes in serum IgG4 levels and the clinical course of patients with IgG4-related ophthalmic disease (IgG4-ROD). (2) Methods: Retrospective analysis of 25 patients with IgG4-ROD. (3) Results: Mean age at diagnosis was 60.68 years. Fifty-six percent of patients had bilateral ocular involvement and 32% had systemic associations. The ocular structures involved were the lacrimal gland (76%), orbital soft tissue (36%), extraocular muscle (20%) and infraorbital nerve (20%). According to last follow-up, 9 (36%) patients had normalized IgG4 levels, and 16 (64%) patients had elevated IgG4 levels. Patients with normalized IgG4 levels had better response to initial steroid treatment and attained a significantly lower IgG4 level after treatment (<i>p</i> = 0.002). The highest IgG4 levels were at baseline and disease recurrence, and lowest after initial treatment. At final follow-up, IgG4 levels differed in patients with remission (mean 326.25 mg/dL) and stable disease (mean 699.55 mg/dL). Subgroup analysis was performed in patients with remission, categorized according to whether IgG4 levels were normalized (9 patients) or elevated (10 patients) on last follow up. The elevated group had a higher percentage of bilateral disease, lacrimal gland involvement and recurrence. (4) Conclusions: IgG4-ROD patients with a greater response to initial steroid therapy were more inclined to have normalized IgG4 levels in the long term. Some patients remained in remission despite persistently elevated IgG4 levels, and had regular follow-up without treatment.https://www.mdpi.com/2075-4426/12/12/1963IgG4-related ophthalmic diseaseIgG4-related diseaseserum IgG4corticosteroids
spellingShingle Wei-Yi Chou
Ching-Yao Tsai
Chieh-Chih Tsai
Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance
Journal of Personalized Medicine
IgG4-related ophthalmic disease
IgG4-related disease
serum IgG4
corticosteroids
title Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance
title_full Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance
title_fullStr Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance
title_full_unstemmed Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance
title_short Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance
title_sort long term follow up in igg4 related ophthalmic disease serum igg4 levels and their clinical relevance
topic IgG4-related ophthalmic disease
IgG4-related disease
serum IgG4
corticosteroids
url https://www.mdpi.com/2075-4426/12/12/1963
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