The Case of IgG4-related Ophthalmic Disease Accompanied by Compressive Optic Neuropathy

Takeishi, Marie; Oshitari, Toshiyuki; Ota, Satomi; Baba, Takayuki; Yamamoto, Shuichi

doi:10.1080/01658107.2017.1389962

Cited by 3 publications

(1 citation statement)

References 6 publications

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“…Oral administration of high-dose glucocorticoids equivalent to IV therapy may not be tolerated due to adverse events, such as gastrointestinal problems and weight gain [27,29]. To the best of our knowledge, IV steroid therapy for IgG4-ROD has been limitedly applied to severe cases with optic nerve dysfunction [30][31][32]. Our results and previous reports suggest that further structured studies are required to evaluate the efficacy of IV steroid therapy for a wider disease spectrum of IgG4-ROD.…”

Section: Plos Onementioning

confidence: 73%

Efficacy and safety of intravenous glucocorticoid therapy for IgG4-related ophthalmic disease

et al. 2023

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Purpose To evaluate and compare the efficacy and safety of intravenous (IV) glucocorticoid therapy with those of oral glucocorticoids as a first-line treatment for IgG4-related ophthalmic disease (IgG4-ROD). Methods We retrospectively reviewed the medical records of patients who underwent systemic glucocorticoid therapy for biopsy-proven IgG4-ROD from June 2012 to June 2022. Glucocorticoids were given either oral prednisolone at an initial dose of 0.6 mg/kg/day for four weeks with subsequent tapering or once weekly IV methylprednisolone (500 mg for six weeks, then 250 mg for six weeks), according to the date of treatment. Clinicoserological features, initial response, relapse during follow-ups, cumulative doses of glucocorticoids, and adverse effects of glucocorticoids were compared for the IV and oral steroid groups. Results Sixty one eyes of 35 patients were evaluated over a median follow-up period of 32.9 months. The complete response rate was significantly higher in the IV steroid group (n = 30 eyes) than in the oral steroid group (n = 31 eyes) (66.7% vs. 38.7%, p = 0.041). Kaplan–Meier analysis showed that the 2-year relapse-free survival was 71.5% (95% confidence interval: 51.6–91.4) and 21.5% (95% confidence interval: 4.5–38.5) in the IV steroid and oral steroid group, respectively (p < 0.001). Although the cumulative dose of glucocorticoids was significantly higher in the IV steroid group than in the oral steroid group (7.8 g vs. 4.9 g, p = 0.012), systemic and ophthalmic adverse effects were not significantly different between the two groups throughout follow-ups (all p > 0.05). Conclusions As a first-line treatment for IgG4-ROD, IV glucocorticoid therapy was well-tolerated, led to better clinical remission and more effectively prevented inflammatory relapse than oral steroids. Further research is needed to establish guidelines on dosage regimens.

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Section: Plos Onementioning

confidence: 73%

Efficacy and safety of intravenous glucocorticoid therapy for IgG4-related ophthalmic disease

et al. 2023

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The 2023 revised diagnostic criteria for IgG4-related ophthalmic disease

Takahira,

Goto,

Azumi

2024

Jpn J Ophthalmol

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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.

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