Rituximab for the treatment of IgG4-related orbital disease: experience from five cases

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dc.contributor.authorWu, A.
dc.contributor.authorAndrew, N.H.
dc.contributor.authorTsirbas, A.
dc.contributor.authorTan, P.
dc.contributor.authorGajdatsy, A.
dc.contributor.authorSelva, D.
dc.date.issued2015
dc.descriptionClinical Study
dc.description.abstractPURPOSE: To review the clinical efficacy and safety of rituximab for treatment of IgG4-related orbital disease (IgG4-ROD). DESIGN: Retrospective multicentre interventional case series. METHODS: Chart review for five cases of biopsy-confirmed IgG4-ROD (IgG4+>10/HPF, ratio of IgG4+/IgG+>40%) treated with rituximab. Information retrieved included the dosing schedule, adverse events and the magnitude, temporality, and duration of the clinical response. RESULTS: All cases of IgG4-ROD were either steroid dependent or steroid resistant. Rituximab doses for induction therapy included two doses of 1000 mg at 2-weekly intervals, and four doses at 375 mg/m(2) at weekly intervals. Two months after starting rituximab, three cases achieved complete clinical resolution and two cases achieved partial clinical resolution. Complete radiological resolution occurred in one case, and partial radiological resolution in three cases. Three cases received rituximab maintenance therapy and one case was commenced on mycophenolate. No relapse occurred during a mean follow-up of 33 months (range: 7-65 months). One disease relapse occurred when the dosing interval of rituximab maintenance therapy was extended to 6-monthly intervals; remission was swiftly achieved with rituximab reinduction therapy. The only adverse effects reported were one episode of fatigue lasting 1 week and two episodes of orbital discomfort. CONCLUSION: Rituximab may be an effective treatment option for IgG4-ROD that is steroid dependent or steroid intolerant. Rituximab therapy resulted in swift clinical and radiological improvement, many months free of relapse, and few side effects.
dc.description.statementofresponsibilityA Wu, N H Andrew, A Tsirbas, P Tan, A Gajdatsy, and D Selva
dc.identifier.citationEye, 2015; 29(1):122-128
dc.identifier.doi10.1038/eye.2014.251
dc.identifier.issn0950-222X
dc.identifier.issn1476-5454
dc.identifier.orcidSelva, D. [0000-0002-2169-5417]
dc.identifier.urihttp://hdl.handle.net/2440/95900
dc.language.isoen
dc.publisherNature Publishing Group
dc.rights© 2015 Macmillan Publishers Limited.
dc.source.urihttps://doi.org/10.1038/eye.2014.251
dc.subjectGlucocorticoids
dc.subjectTomography, X-Ray Computed
dc.subjectOrbital Pseudotumor
dc.subjectAntibodies, Monoclonal, Murine-Derived
dc.titleRituximab for the treatment of IgG4-related orbital disease: experience from five cases
dc.typeJournal article
pubs.publication-statusPublished

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