The 2023 revised diagnostic criteria for IgG4-related ophthalmic disease

Abstract

Immunoglobulin G4 (IgG4)-related disease is a clinical entity characterized by elevated serum IgG4 concentrations and infiltration of IgG4-immunopositive plasmacytes in various organs, including ophthalmic lesions. Diagnostic criteria for IgG4-related ophthalmic disease (IgG4-ROD) were established in 2014 and describe the most affected ocular adnexal tissues such as lacrimal glands, trigeminal nerves and extraocular muscles, but do not mention optic neuropathy, the most severe indication of ophthalmic lesions. We reviewed published case reports of optic neuropathy in IgG4-related disease (n = 44), and in many cases, decreased visual acuities recovered well following treatment such as systemic corticosteroids, rituximab, and orbital surgery. However, some patients did not recover, especially when pretreatment visual acuities were as low as light perception or less. Herein, we propose a 2023 revised diagnostic criteria for IgG4-ROD, which include a reminder not to overlook optic neuropathy. The 2014 diagnostic criteria specify mucosa-associated lymphoid tissue (MALT) lymphoma as an important differential diagnosis for the relationship between IgG4-ROD and orbital lymphoma. The 2023 revision directs physicians' attention toward lymphomas other than MALT lymphoma, considering that the 2014 criteria might have placed too much emphasis on MALT lymphoma.

Keywords: IgG4-related disease; IgG4-related ophthalmic disease; Lacrimal gland; MALT lymphoma; Optic neuropathy.

Fig. 1

A case of optic neuropathy in IgG4-related disease treated by steroid pulse therapy. a) Magnetic resonance imaging (MRI) before therapy shows bilateral extraocular muscle swelling and mass lesions at the orbital apex (arrows) compressing the optic nerves. b) After the first course (3 days) of steroid pulse therapy, the left visual field presents central scotoma (visual acuity 0.5). c) After the second course of steroid pulse therapy, the left visual field shows only paracentral scotoma (visual acuity 0.8). d) MRI after intravenous and oral steroid therapy shows that the orbital apex mass lesions disappeared (arrows)