Immunoglobulin G4-positive Sclerosing Idiopathic Orbital Inflammation: New Neuro-ophthalmological Presentations

sight-threatening optic nerve compression or when vital organ function is compromised. Chemotherapeutic agents generally consist of methotrexate, vincristine, azathioprine, cyclophosphamid, antitumour necrosis factor alpha, and anti-CD20 monoclonal antibody (rituximab). Radiation therapy has been successfully used in few cases with a total dose of 30 Gray. 10 No conclusive role of these various treatments was found in the literature.

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The treatment outcomes in IgG4‐related orbital disease: a systematic review of the literature

Karim

Verdijk

Hagen

et al. 2019

Acta Ophthalmologica

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IgG4‐related disease (IgG4‐RD) is an immune‐mediated systemic fibro inflammatory disease. Treatment of IgG4‐related orbital disease (IgG4‐ROD) is often indicated to relieve the symptoms and to prevent complications. For IgG4‐ROD, no international formal treatment guidelines are available and the optimal treatment strategy is uncertain. In this systematic review, we describe the efficacy of conventional and biologic disease‐modifying antirheumatic drugs (DMARDs) in IgG4‐ROD. A systematic search of Embase, Medline, Web‐of‐Science, PubMed publisher, Cochrane and Google Scholar was performed for treatment outcomes in IgG4‐ROD. Relevant articles on treatment of IgG4‐ROD were retrieved to last date of inclusion 3 January 2018. The following inclusion criteria were used: articles in English or English translation, studies evaluating the use of DMARDs (conventional and biologic) in the treatment of IgG4‐ROD. Meta‐analysis and review articles were excluded. A final selection after full‐text evaluation was made by independent reviewers, based on treatment of IgG4‐ROD with DMARDs and the availability of treatment outcomes. With this systematic review, we identified 35 studies and case reports/series on IgG4‐ROD, describing 95 patients, treated with conventional and/or biologic DMARDs. The success of conventional DMARDs varies between 36% and 75% in patients with IgG4‐ROD, while rituximab is successful in the majority (93%) of the patients. Based on this systematic review, rituximab is the most effective DMARD in IgG4‐ROD, while the efficacy of conventional DMARDs is limited. We propose early initiation of rituximab in case of refractory and organ‐ or life‐threatening disease.

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Immunoglobulin G4-positive Sclerosing Idiopathic Orbital Inflammation: New Neuro-ophthalmological Presentations

Cited by 3 publications

References 33 publications

Update on Treatment of Idiopathic (and Non-Idiopathic) Orbital Inflammation

Update on Treatment of Idiopathic (and Non-Idiopathic) Orbital Inflammation

Compressive optic neuropathy in non-thyroidal lymphocytic infiltrative disorders of the extraocular muscles

The treatment outcomes in IgG4‐related orbital disease: a systematic review of the literature

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