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

Sanne E Detiger, A Faiz Karim, Robert M Verdijk, P Martin van Hagen, Jan A M van Laar, Dion Paridaens

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Pages (from-to)451-459
Number of pages9
JournalActa Ophthalmologica
Issue number5
Publication statusPublished - Aug 2019


  • Antirheumatic Agents/therapeutic use
  • Biological Factors/therapeutic use
  • Humans
  • Immunoglobulin G4-Related Disease/immunology
  • Orbital Diseases/immunology
  • Practice Guidelines as Topic
  • Remission Induction/methods
  • Treatment Outcome


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