Electroretinographic improvement after rituximab therapy in a patient with autoimmune retinopathy

Abstract

PurposeTo describe the effect of rituximab on full-field electroretinography (ERG) in a patient with nonparaneoplastic autoimmune retinopathy (npAIR).ObservationsA 58-year-old male patient with visual complaints, positive anti-retinal antibodies and negative work-up for cancer was diagnosed with npAIR. Visual acuity and ancillary tests were normal except abnormal ERG in both eyes. The patient was given one course of rituximab 375 mg/m2/week for 4 weeks and cyclophosphamide 1 gr/m2/month for 6 months. A second course of rituximab was necessary as autoantibody titers showed no change and as new antibodies were noted after treatment with rituximab and cyclophosphamide. Electroretinography was repeated after the first course of rituximab, after cyclophosphamide, and the second course of rituximab therapy.Conclusions and importanceRituximab therapy led to marked improvement in full-field ERG readings and regression of symptoms was reported by the patient after rituximab infusions. The effect of rituximab in npAIR was objectively demonstrated with ERG.

Unilateral paraneoplastic optic disc edema and retinal periphlebitis in pineal germinoma

“…azathioprine, mycophenolate mofetil, cyclosporine A and rituximab) are used to treat CAR. 5 , 7 , 9 We emphasize, that treatment of pineal germinoma resulted in complete resolution of the paraneoplastic syndrome in our patient as in previously reported cases and that there was no need for treatment of ocular involvement as in CAR.…”

“…Cancer associated retinopathy (CAR) is a well-recognized ocular paraneoplastic syndrome which is characterized by progressive vision loss, abnormal electroretinography (ERG), visual field deficits, and presence of circulating anti-retinal autoantibodies. 5 , 7 , 8 , 9 , 10 In CAR autoimmunity directed against retinal antigens usually leads to bilateral retinal symptoms and signs although asymmetric between the eyes. 7 , 8 , 9 However, there are two separate case reports showing unilateral involvement in CAR.…”

“… 5 , 7 , 8 , 9 , 10 In CAR autoimmunity directed against retinal antigens usually leads to bilateral retinal symptoms and signs although asymmetric between the eyes. 7 , 8 , 9 However, there are two separate case reports showing unilateral involvement in CAR. 11 , 12 Roels and associates reported a case with unilateral visual symptoms consistent with CAR, abnormal unilateral ERG, and positive autoantibodies against TRPM1.…”

“… 4 The authors were also able to document resolution of antibodies against 35kDa optic nerve protein along with inflammatory fundus findings after successful treatment of pineal germinoma. 4 Although we search for anti-retinal and anti-optic nerve antibodies to diagnose and to survey response to therapy in autoimmune retinopathies in our clinical routine, 9 the patient reported herein did not undergo such testing because of economic reasons.…”

An update on autoimmune retinopathy

“…There is no standard treatment protocol currently but the various therapeutic options described for AIRs, in the previous literature[ 7 10 21 22 23 39 40 51 59 60 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 ] are (1) immunosuppression through systemic and/or topical (intravitreal/sub-tenon/depot) corticosteroids, (2) immunomodulators like cyclosporine (calcineurin inhibitor which prevents IL-2 transcription), infliximab (anti-TNFα antibody), mycophenolate mofetil (IMP dehydrogenase inhibitor, preventing purine synthesis), azathioprine (purine antimetabolite), (3) biologics such as monoclonal antibodies like rituximab (anti-CD20 antibody), alemtuzumab (anti-CD52 antibody), ipilimumab (antagonist antibody against cytotoxic T-lymphocyte antigen-4), tocilizumab (anti-IL-6 receptor antibody), sarilumab (anti-IL-6 receptor antibody), (4) and others like intravenous immunoglobulin (IVIG), plasmapheresis. IVIG has been hypothesized to have several mechanisms of action like neutralization of autoantibodies, binding of complement components, inhibition of dendritic cells maturation, modulation of intercellular adhesion, and contribution of IgG4.…”