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-ofScience, 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 organor life-threatening disease.

Additional Metadata
Keywords disease-modifying antirheumatic drugs – IgG4-related disease – orbit – rituximab
Persistent URL dx.doi.org/10.1111/aos.14048, hdl.handle.net/1765/118902
Journal Acta Ophthalmologica
Citation
Detiger, S.E., Karim, A.F., Verdijk, R.M, van Hagen, M.P, van Laar, J.A.M, & Paridaens, D. (2019). The treatment outcomes in IgG4-related orbital disease: a systematic review of the literature. Acta Ophthalmologica, 97(5), 451–459. doi:10.1111/aos.14048