Rituximab as a monotherapy or in combination therapy for the treatment of non-paraneoplastic autoimmune retinopathy

Maleki, Arash; Lamba, Neerav; Ma, Lina; Lee, Stacey; Schmidt, Alexander; Foster, C. Stephen

doi:10.2147/opth.s120162

Cited by 23 publications

(25 citation statements)

References 26 publications

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“…Additionally, one case series studied six npAIR patients receiving rituximab and/or combination therapy [19]. They found that following mono- or combinatorial therapy, 66.7% of eyes had stable visual acuity, 50% showed stability on visual field testing, and 33.3% showed stability or improvement on ERG.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of rituximab in non-paraneoplastic autoimmune retinopathy

Boudreault

Justus

Sengillo

et al. 2017

Orphanet J Rare Dis

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BackgroundAutoimmune retinopathy (AIR) is a rare but potentially blinding condition that is often underdiagnosed. Common features in AIR presentation include rapidly progressive vision loss with abnormal electrophysiological responses of the retina associated with positive anti-retinal antibodies. AIR is also challenging to treat, and thus, the introduction of new potential therapeutic agents is welcomed. The goal of this communication is to assess the effects of rituximab infusions on electroretinogram (ERG) responses and visual function outcomes in patients with non-paraneoplastic autoimmune retinopathy (npAIR).ResultsFollowing infusion(s), three out of five patients showed no evidence of disease progression or improved, while two patients continued to progress on ERG. One patient demonstrated improvement in visual acuity (2 lines) in both eyes. ERG responses provided objective monitoring of patients’ visual function and response to immunosuppression over time.ConclusionsThese findings suggest that patients with npAIR unresponsive to other immunosuppression therapies may benefit from rituximab infusion, although stabilization rather than improvement was more frequently the outcome in our case series. Furthermore, regularly scheduled ERG follow-up examinations are recommended for monitoring patients’ progression during treatment.

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Section: Discussionmentioning

confidence: 99%

Efficacy of rituximab in non-paraneoplastic autoimmune retinopathy

Boudreault

Justus

Sengillo

et al. 2017

Orphanet J Rare Dis

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“…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.…”

Section: Treatmentmentioning

confidence: 99%

“…Multiple case series[ 82 85 86 ] and case reports[ 71 75 76 80 81 87 93 ] in the recent years describe the potential use of rituximab for improvement in retinal anatomy, function, and an overall good response, in both CAR and npAIR. A retrospective case series of 16 AIR patients (one MAR, six CAR, and nine npAIR) who underwent treatment with rituximab, along with concomitant immunosuppressive agents (mycophenolate/cyclophosphamide/IVIG/bortezomib/topical steroids), demonstrated 77% of eyes having stable or improved visual outcomes.…”

Section: Treatmentmentioning

confidence: 99%

“…[ 85 ] Yet another case series of six patients with npAIR, who received rituximab and/or combination treatment, reported stabilization of visual acuity in two-thirds of them and overall improvement of visual fields or ERG parameters in 75% of patients. [ 86 ] The potential benefit of alemtuzumab (30 mg IV three times a week for 4 months) has also been described in a CAR patient with up to 8 years of follow-up. [ 67 ]…”

Section: Treatmentmentioning

confidence: 99%

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An update on autoimmune retinopathy

Majumder

Marchese

Pichi

et al. 2020

Indian J Ophthalmol

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Autoimmune retinopathy (AIR) refers to a group of rare autoimmune retinal degenerative diseases presumably caused by cross-reactivity of serum autoantibodies against retinal antigens. The pathogenesis of AIR remains largely presumptive and there are a significant number of antiretinal antibodies that have been detected in association with AIR. The diagnosis of AIR is largely based on the demonstration of antiretinal antibodies in the serum along with suggestive clinical features and ancillary investigations. A high index of suspicion along with early diagnosis and treatment may play a critical role to lower the risk of irreversible immunological damage to the retinal cells in these patients. A multi-disciplinary approach for complete management and evaluation is helpful in such conditions. Various therapeutic options have been described for the treatment of AIR, though there is no consensus on standard treatment protocol.

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“…The number of AR patients benefited from rituximab treatment ( 128 – 131 ). The efficacy of rituximab was studied in six patients (12 eyes) administrated as a monotherapy or in combination therapy ( 132 ). Some patients in the study had anti-enolase (three patients) and anti-aldolase (one patient) AAbs.…”

Section: Management Of Autoimmune Retinopathiesmentioning

confidence: 99%

Impact of Autoantibodies against Glycolytic Enzymes on Pathogenicity of Autoimmune Retinopathy and Other Autoimmune Disorders

Adamus

2017

Front. Immunol.

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Autoantibodies (AAbs) against glycolytic enzymes: aldolase, α-enolase, glyceraldehyde-3-phosphate dehydrogenase, and pyruvate kinase are prevalent in sera of patients with blinding retinal diseases, such as paraneoplastic [cancer-associated retinopathy (CAR)] and non-paraneoplastic autoimmune retinopathies, as well as in many other autoimmune diseases. CAR is a degenerative disease of the retina characterized by sudden vision loss in patients with cancer and serum anti-retinal AAbs. In this review, we discuss the widespread serum presence of anti-glycolytic enzyme AAbs and their significance in autoimmune diseases. There are multiple mechanisms responsible for antibody generation, including the innate anti-microbial response, anti-tumor response, or autoimmune response against released self-antigens from damaged, inflamed tissue. AAbs against enolase, GADPH, and aldolase exist in a single patient in elevated titers, suggesting their participation in pathogenicity. The lack of restriction of AAbs to one disease may be related to an increased expression of glycolytic enzymes in various metabolically active tissues that triggers an autoimmune response and generation of AAbs with the same specificity in several chronic and autoimmune conditions. In CAR, the importance of serum anti-glycolytic enzyme AAbs had been previously dismissed, but the retina may be without pathological consequence until a failure of the blood–retinal barrier function, which would then allow pathogenic AAbs access to their retinal targets, ultimately leading to damaging effects.

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Rituximab as a monotherapy or in combination therapy for the treatment of non-paraneoplastic autoimmune retinopathy

Cited by 23 publications

References 26 publications

Efficacy of rituximab in non-paraneoplastic autoimmune retinopathy

Efficacy of rituximab in non-paraneoplastic autoimmune retinopathy

An update on autoimmune retinopathy

Impact of Autoantibodies against Glycolytic Enzymes on Pathogenicity of Autoimmune Retinopathy and Other Autoimmune Disorders

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