IgG4-related disease presenting as recurrent scleritis combined with optic neuropathy.

Published on Jan 5, 2021in BMC Ophthalmology1.413
· DOI :10.1186/S12886-020-01774-6
Su Jin Kim2
Estimated H-index: 2
(PNU: Pusan National University),
Seung Uk Lee5
Estimated H-index: 5
(Kosin University)
+ 4 AuthorsJi Eun Lee4
Estimated H-index: 4
(PNU: Pusan National University)
BACKGROUND We report a case of atypical presentation of IgG4-related disease (IgG4-RD) with recurrent scleritis and optic nerve involvement. CASE PRESENTATION A 61-year-old male presented with ocular pain and injection in his left eye for 2 months. Ocular examination together with ancillary testing led to the diagnosis of scleritis, which relapsed in spite of several courses of steroid treatment. After cessation of steroid, the patient complained of severe retro-orbital pain and blurred vision. His best corrected vision was count finger, the pupil was mid-dilated and a relative afferent pupillary defect was found. Funduscopic examination demonstrated disc swelling. Magnetic resonance imaging (MRI) showed enhancing soft tissue encasing the left globe, medial rectus muscle and optic nerve. Systemic work-up revealed multiple nodules in right lower lung and a biopsy showed histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms with no recurrence for 2 years. CONCLUSIONS This case highlights the significance of IgG4-RD in the differential diagnosis of recurrent scleritis. IgG4-RD may cause optic neuropathy resulting in visual loss. Early diagnosis and proper treatment can prevent irreversible organ damage and devastating visual morbidity.
#1Jing Li (Capital Medical University)H-Index: 2
#2Yan ZhangH-Index: 1
Last. Hongyang Li (Capital Medical University)H-Index: 1
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Background The following study investigates the involvement of optic neuropathy in IgG4-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4-ROD related optic neuropathy related to other orbital diseases.
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IgG4-related disease is a multi-organ fibro-inflammatory disease with characteristic histopathology showing lymphoplasmacytic infiltration, increased IgG4+ plasma cells and elevated IgG4/IgG ratios (>40%). The lacrimal gland is the most common ocular site of involvement. Scleritis and intraocular involvement in IgG4-related ophthalmic disease (IgG4-ROD) have recently been reported. The purpose of this review is to describe orbital and intraocular IgG4-ROD with a focus on publications since 2016....
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#1Faiz Karim (EUR: Erasmus University Rotterdam)H-Index: 4
#2Joeri de Hoog (EUR: Erasmus University Rotterdam)H-Index: 4
Last. Jan A. M. van Laar (EUR: Erasmus University Rotterdam)H-Index: 12
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A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with co...
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#1Gyuhwan Hwang (SCH: Soonchunhyang University)H-Index: 1
#2So-Young Jin (SCH: Soonchunhyang University)H-Index: 13
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#1Elise PhilippakisH-Index: 1
#2Nathalie Cassoux (Curie Institute)H-Index: 36
Last. Valérie TouitouH-Index: 23
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13 CitationsSource
PURPOSE: To review the current state of knowledge of IgG4-related ophthalmic disease (IgG4-ROD). METHODS: A review of the literature and personal experience of the authors. RESULTS: IgG4-related disease is a recently recognized fibroinflammatory disorder that may affect 1 or more organs. It is characterized by lymphoplasmacytic infiltrates with large numbers of IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration as well as peripheral eosinophilia, ...
43 CitationsSource
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#1John H. Stone (Harvard University)H-Index: 76
#2John K.C. ChanH-Index: 45
Last. Yoh ZenH-Index: 69
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