{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T06:26:49Z","timestamp":1761546409867,"version":"build-2065373602"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T00:00:00Z","timestamp":1761523200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1652730","type":"journal-article","created":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T06:21:34Z","timestamp":1761546094000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: IgG4-related ophthalmic disease presenting with unilateral proptosis"],"prefix":"10.3389","volume":"16","author":[{"given":"Xiaodong","family":"Li","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xuewei","family":"Qin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mei","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xi","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,27]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1038\/s41584-020-0500-7","article-title":"IgG4-related disease:an update on pathophysiology and implications for clinical care","volume":"16","author":"Perugino","year":"2020","journal-title":"Nat Rev Rheumatol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1007\/s10384-024-01072-4","article-title":"The 2023 revised diagnostic criteria for IgG4-related ophthalmic disease","volume":"68","author":"Takahira","year":"2024","journal-title":"Jpn J Ophthalmol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1007\/s10384-021-00847-3","article-title":"Clinical features and symptoms of IgG4-related ophthalmic disease:a multicenter study","volume":"65","author":"Goto","year":"2021","journal-title":"Jpn J Ophthalmol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1007\/s10384-014-0352-2","article-title":"Diagnostic criteria for IgG4-related ophthalmic disease","volume":"59","author":"Goto","year":"2015","journal-title":"Jpn J Ophthalmol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3760\/cma.j.cn112137-20240814-01870","article-title":"Expert consensus on the diagnosis and treatment of immunoglobulin-G4 related ophthalmic disease (2024 edition)","volume":"104","author":"Yi","year":"2024","journal-title":"Zhonghua Yi Xue Za Zhi"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1038\/s41584-020-0500-7","article-title":"IgG4-related disease: an update on pathophysiology and implications for clinical care","volume":"16","author":"Perugino","year":"2020","journal-title":"Nat Rev Rheumatol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1038\/s41584-025-01240-x","article-title":"IgG4-related disease and other fibro-inflammatory conditions","volume":"21","author":"Peyronel","year":"2025","journal-title":"Nat Rev Rheumatol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"rxaf015","DOI":"10.1093\/mrcr\/rxaf015","article-title":"Successful glucocorticoid treatment case for IgG4-related long-standing optic neuropathy","author":"Fukami","year":"2025","journal-title":"Mod Rheumatol Case Rep"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1080\/01658107.2017.1389962","article-title":"The case of igG4-related ophthalmic disease accompanied by compressive optic neuropathy","volume":"42","author":"Takeishi","year":"2018","journal-title":"Neuroophthalmology"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1038\/s41433-020-1083-x","article-title":"Clinical features and outcomes of IgG4-related idiopathic orbital inflammatory disease: from a large southern China-based cohort","volume":"35","author":"Chen","year":"2020","journal-title":"Eye (Lond)"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1097\/IOP.0000000000001975","article-title":"Clinical outcomes of igG4-related ophthalmic disease and idiopathic sclerosing orbital inflammation","volume":"38","author":"Son","year":"2022","journal-title":"Ophthalmic Plast Reconstr Surg"},{"key":"B12","doi-asserted-by":"publisher","first-page":"0","DOI":"10.1097\/WNO.0000000000000193","article-title":"IgG4-related disease: a neuro-ophthalmological perspective","volume":"34","author":"Satoshi","year":"2014","journal-title":"J Neuroophthalmol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"6668510","DOI":"10.1155\/2021\/6668510","article-title":"o explore MR imaging radiomics for the differentiation of orbital lymphoma and igG4-related ophthalmic disease","volume":"2021","author":"Yuan","year":"2021","journal-title":"BioMed Res Int"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1097\/IOP.0000000000000363","article-title":"IgG4-related ophthalmic disease. 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