Immunoglobulin G4-related ophthalmic disease presenting as a steroid-resistant choroidal mass: a case report
Keywords:Choroid diseases, Immunoglobulin G4-related disease, Scleritis, Uveitis
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.
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