PurposeTo review the clinical efficacy and safety of rituximab for treatment of IgG4-related @entity2694 (IgG4-ROD).DesignRetrospective multicentre interventional case series.MethodsChart 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.ResultsAll cases of IgG4-ROD were either @entity134 dependent or @entity134 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 @entity6051 . 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 @entity1295 lasting 1 week and two episodes of orbital discomfort.ConclusionRituximab may be an effective treatment option for IgG4-ROD that is @entity134 dependent or @entity134 intolerant. Rituximab therapy resulted in @entity514 clinical and radiological improvement, many months free of relapse, and few side effects.Eye advance online publication, 24 October 2014; doi:10.1038/eye.2014.251.

Rituximab for the treatment of IgG4-related XXXX : experience from five cases.

@entity514 :: ('8895', 'Species') :: ['swift'] @entity1295 :: ('MESH:D005221', 'Disease') :: ['fatigue'] @entity2694 :: ('MESH:D009916', 'Disease') :: ['orbital disease'] @entity134 :: ('CHEBI:35341', 'Chemical') :: ['steroid'] @entity6051 :: ('CHEBI:168396', 'Chemical') :: ['mycophenolate']
@entity2694 :: (MESH:D009916,Disease) :: ['orbital disease']
