Case Report: IgG4-related ophthalmic disease presenting with unilateral proptosis

IgG4-related eye disease (IgG4-ROD) is characterized by diffuse swelling or well-defined masses in the lacrimal glands, extraocular muscles, and eyelids, often accompanied by eyelid swelling, proptosis, and diplopia. It typically affects both sides symmetrically and can mimic various infectious, inflammatory, and neoplastic diseases. Early recognition and steroid therapy can significantly improve symptoms, but recurrence is common, necessitating careful and long-term follow-up. B-cell depletion therapy, such as rituximab, is increasingly being incorporated into standard treatment protocols. This article reports a case of IgG4-ROD misdiagnosed as a unilateral orbital mass, summarizing its clinical features, diagnostic approach, and treatment outcomes. Combined with a literature review and analysis, this study aims to provide reference for the clinical identification of IgG4-ROD.

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Publication Details

Journal
Frontiers in Immunology
Published
2025-10-27
DOI
https://doi.org/10.3389/fimmu.2025.1652730
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
Field-Weighted Citation Impact
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article

Case Report: IgG4-related ophthalmic disease presenting with unilateral proptosis

Zheng Xi, Mei Chen, Xiaodong Li, Xuewei Qin
Frontiers in Immunology
IgG4-Related and Inflammatory Diseases
article

Case Report: IgG4-related ophthalmic disease presenting with unilateral proptosis

Zheng Xi, Mei Chen, Xiaodong Li, Xuewei Qin
article en

Abstract

IgG4-related eye disease (IgG4-ROD) is characterized by diffuse swelling or well-defined masses in the lacrimal glands, extraocular muscles, and eyelids, often accompanied by eyelid swelling, proptosis, and diplopia. It typically affects both sides symmetrically and can mimic various infectious, inflammatory, and neoplastic diseases. Early recognition and steroid therapy can significantly improve symptoms, but recurrence is common, necessitating careful and long-term follow-up. B-cell depletion therapy, such as rituximab, is increasingly being incorporated into standard treatment protocols. This article reports a case of IgG4-ROD misdiagnosed as a unilateral orbital mass, summarizing its clinical features, diagnostic approach, and treatment outcomes. Combined with a literature review and analysis, this study aims to provide reference for the clinical identification of IgG4-ROD.

Frontiers in ImmunologyVol. 16
Guiyang College of Traditional Chinese Medicine (CN), Affiliated Hospital of Guizhou Medical University (CN), Shanghai Traditional Chinese Medicine Hospital (CN)
Openalex Percentile: Top 56%
IgG4-Related and Inflammatory Diseases
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