Immunoglobulin G4-related ophthalmic disease presenting as a steroid-resistant choroidal mass: a case report

Authors

  • Cherie Yee-Kiu Wong Hong Kong Eye Hospital https://orcid.org/0000-0002-1543-4640
  • Moon-ho Leung
  • Vanissa WS Chow
  • Shaheeda Mohamed
  • N M Lam
  • Hunter KL Yuen
  • Carmen KM Chan

Keywords:

Choroid diseases, Immunoglobulin G4-related disease, Scleritis, Uveitis

Abstract

Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) is a cause for orbital and ocular inflammation. Involvement of the sclera and intraocular tissues is less common, and presentation is mostly unilateral. We hereby describe a probable case of steroid-resistant intraocular IgG4-ROD presenting as a choroidal mass in the left eye, with sequential anterior uveitis and scleritis of the fellow right eye. The diagnostic path was not straightforward and included subconjunctival biopsy, blood tests, magnetic resonance imaging, positron emission tomography–computed tomography, and biopsies in two extra-ocular sites. Disease progression was rapid in the left eye despite high-dose oral steroids, while the inflammation of the fellow eye was controlled with the use of rituximab and second-line immunosuppressants. At the 3-year follow-up, the ocular inflammation was well controlled. The left eye was phthsical, but the right eye maintained a visual acuity of 20/20.

References

Derzko-Dzulynsky L. IgG4-related disease in the eye and ocular adnexa. Curr Opin Ophthalmol 2017;28:617-22.

Ohno K, Sato Y, Ohshima K, et al. IgG4-related disease involving the sclera. Mod Rheumatol 2014;24:195-8.

Goto H, Ueda S. Immunoglobulin G4-related ophthalmic disease involving the sclera misdiagnosed as intraocular tumor: report of one case. Ocul Oncol Pathol 2016;2:285-8.

Reynolds GL, Norris JH, Aslam S, Sharma S. IgG4-related disease presenting as posterior scleritis and vitritis, progressing to multifocal orbital involvement. BMJ Case Rep 2017;2017:bcr2017219568.

Gange WS, Holland SM, De Alba F. IgG4-related ophthalmic disease presenting as choroidal and orbital lesions. Retin Cases Brief Rep 2019;13:283-6.

Aragona E, Miserocchi E, Arrigo A, et al. Immunoglobulin G4-related ophthalmic disease mimicking intraocular lymphoma: a case report. Retin Cases Brief Rep 2022;16:32-5.

Attiku Y, Rishi P, Biswas J, Krishnakumar S. Intraocular IgG4 disease masquerading as nodular scleritis. Indian J Ophthalmol 2020;68:1935-6.

Koenigstein D, Neudorfer M, Goldenberg D, Habot-Wilner Z. Choroidal effusion as an ocular manifestation of immunoglobulin G4-related disease. Retin Cases Brief Rep 2016;10:197-200.

Goto H, Takahira M, Azumi A; Japanese Study Group for IgG4-Related Ophthalmic Disease. Diagnostic criteria for IgG4-related ophthalmic disease. Jpn J Ophthalmol 2015;59:1-7.

Philippakis E, Cassoux N, Charlotte F, et al. IgG4-related disease masquerading as recurrent scleritis and chronic conjunctivitis. Ocul Immunol Inflamm 2015;23:168-72.

Löhr JM, Beuers U, Vujasinovic M, et al. European Guideline on IgG4-related digestive disease: UEG and SGF evidence-based recommendations. United European Gastroenterol J 2020;8:637-66.

Khosroshahi A, Wallace ZS, Crowe JL, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol 2015;67:1688-99.

Wallace ZS, Mattoo H, Mahajan VS, et al. Predictors of disease relapse in IgG4-related disease following rituximab. Rheumatology (Oxford) 2016;55:1000-8.

Yu T, Wu Y, Liu J, Zhuang Y, Jin X, Wang L. The risk of malignancy in patients with IgG4-related disease: a systematic review and meta-analysis. Arthritis Res Ther 2022;24:14.

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Published

2022-12-29

How to Cite

1.
Wong CY-K, Leung M- ho, Chow VW, Mohamed S, Lam NM, Yuen HK, Chan CK. Immunoglobulin G4-related ophthalmic disease presenting as a steroid-resistant choroidal mass: a case report. Hong Kong J Ophthalmol [Internet]. 2022Dec.29 [cited 2023Feb.3];26(2). Available from: https://hkjo.hk/index.php/hkjo/article/view/334

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Section

Case Report