Rituximab in refractory myasthenia gravis: Extended prospective study results

Beecher, Grayson; Anderson, Dustin; Siddiqi, Zaeem A.

doi:10.1002/mus.26156

Cited by 56 publications

(54 citation statements)

References 19 publications

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“…Our study showed that CD19 + and CD27 + B cells decreased markedly during the first 3 months. Previous studies demonstrated that antibody titers, NK cells, and CD19 + or CD20 + B cells may not be useful in predicting relapses . However, CD27 + memory B cells may be an effective marker, as shown by a comparative study in which rituximab was administered to patients with refractory MG. CD19 + CD27 + memory B‐cell recovery, but not total CD19 + recovery, was associated with the relapse of clinical symptoms in all patients.…”

Section: Discussionmentioning

confidence: 99%

“…Previous studies demonstrated that antibody titers, NK cells, and CD19 + or CD20 + B cells may not be useful in predicting relapses. 6,26,27 However, CD27 + memory B cells may be an effective marker, as shown by a comparative study 28 in which rituximab was administered to patients with refractory MG. CD19 + CD27 + memory B-cell recovery, but not total CD19 + recovery, was F I G U R E 3 Changes in lymphocyte subgroups during rituximab treatment (regression curves were generated via the LOESS method; the shadow represents 95% confidence interval around each curve). During rituximab treatment, B cells decreased markedly in the first 3 months and remained at low levels.…”

Section: Discussionmentioning

confidence: 99%

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Low‐dose rituximab every 6 months for the treatment of acetylcholine receptor–positive refractory generalized myasthenia gravis

et al. 2020

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Introduction In this prospective, open‐label study we explore the effectiveness of low‐dose rituximab every 6 months in treating refractory generalized myasthenia gravis (GMG). Methods Twelve patients with acetylcholine receptor (AChR)‐positive refractory GMG were enrolled for the study. The primary endpoint was the change in quantitative myasthenia gravis (QMG) score from baseline to the study end. Secondary endpoints included changes in manual muscle testing (MMT), MG‐Related Activities of Daily Living (MG‐ADL), and 15‐item Quality‐of‐Life (MGQOL‐15) scores, as well as prednisolone reduction. Results MG decreased from 18.25 ± 4.03 to 8.42 ± 3.99 (P = .0001), MMT from 27.50 ± 17.78 to 4.58 ± 4.34 (P = .0001), ADL from 8.50 ± 2.84 to 1.17 ± 1.27 (P < .0001), MGQOL‐15 from 37.25 ± 13.78 to 17.50 ± 9.73 (P = .0015), and prednisolone dose from 29.38 ± 11.92 mg/day to 8.86 ± 1.88 mg/day (P ≤ .01). Discussion Low‐dose rituximab every 6 months is effective in treating refractory GMG patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Low‐dose rituximab every 6 months for the treatment of acetylcholine receptor–positive refractory generalized myasthenia gravis

et al. 2020

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“…Online supplementary file 1 summarises data from selected studies on the use of RTX in AChR-MG. Thirteen studies have been selected, showing good safety 1 2 6 7 9 11–18. The data collected from each report included the number and regimen of infusions used for therapy with RTX, antibody type and titres pretreatment and post-treatment, follow-up duration, data on safety and postintervention status of MG.…”

Section: Resultsmentioning

confidence: 99%

“…The data obtained over the last few years on the use of RTX in refractory MG are heterogeneous in terms of posology, administration scheme and patients’ evaluation. However, previous studies have shown that RTX is safe, since the frequency of serious adverse events is low 2 6–10. The most common side effects are related to the infusion and can be easily prevented by pretreatment with steroids and antihistamines 1 2 9.…”

Section: Discussionmentioning

confidence: 99%

“…Although many immunosuppressive and immunomodulatory therapies are available, including steroids, azathioprine, mycophenolate mofetil, methotrexate and cyclosporine, up to 20% of patients with MG are refractory to conventional treatment, showing incomplete responses to multiple combined therapies or requiring repeated immunoglobulin (IVIg) infusions or plasma exchange 6 7. Refractory MG decreases the quality of life of affected patients, and sometimes, it requires hospitalisation for potentially lethal exacerbations 8.…”

Section: Introductionmentioning

confidence: 99%

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Rituximab in AChR subtype of myasthenia gravis: systematic review

Stefano

Lupica

Rispoli

et al. 2020

J Neurol Neurosurg Psychiatry

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Myasthenia gravis (MG) is a chronic autoimmune disorder of the neuromuscular junction characterised by an autoantibody against acetylcholine receptor (AChR-Ab), autoantibody against muscle-specific kinase (MuSK-Ab), lipoprotein-related protein 4 or agrin in the postsynaptic membrane at the neuromuscular junction. Many patients are resistant to conventional treatment and effective therapies are needed. Rituximab (RTX) is a monoclonal antibody directed against CD20 antigen on B cells which has been successfully employed in anti-MuSK-Ab+MG, but the efficacy in anti-AChR-Ab+MG is still debated. The purpose of this systematic review was to describe the best evidence for RTX in the acetylcholine receptor subtype. The authors undertook a literature search during the period of 1999–2019 according to the Preferred Reporting Items for Systematic Reviews and Meta-Analys methodology, employing (myasthenia)+(gravis)+(RTX) as search terms. The analysis was confined to studies that include at least five patients with confirmed anti-AChR-Ab+MG. Thirteen studies have been selected, showing a good safety. The data obtained were heterogeneous in terms of posology, administration scheme and patients’ evaluation, ranging from a minimum of two to a maximum of three cycles. RTX led to a sustained clinical improvement with prolonged time to relapse, in parallel to a reduction or discontinuation of other immunosuppressive therapies. Treatment with RTX appears to work in some but not all patients with anti-AChR-Ab+MG, but randomised controlled trials are needed. Future studies should take into account the subtype of MG and employ reliable measures of outcome and severity focusing on how to identify patients who may benefit from the treatment. Trial registration number: NCT02110706.

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The use of eculizumab in ventilator‐dependent myasthenia gravis patients

Usman

Chrisman²,

Houston

et al. 2021

Muscle and Nerve

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Introduction/Aims Eculizumab has been shown to be efficacious in acetylcholine receptor antibody–positive (AChR+) Myasthenia Gravis Foundation of America (MGFA) class II, III, and IV generalized myasthenia gravis (gMG) patients. However, it has not been studied in MGFA class V gMG patients. Methods We report three AChR+, refractory, MGFA class V gMG patients treated with eculizumab. MGFA class, MG‐Composite (MGC) score and MG Activities of Daily Living (MG‐ADL) score were assessed before and after eculizumab. Results Two of three gMG patients, refractory to intravenous immunoglobulin, plasmapheresis, prednisone, and (in one case) rituximab, showed a robust response to eculizumab with marked improvement in MGFA, MG‐ADL, and MGC measures. The third patient showed a partial response to eculizumab but remained on noninvasive ventilation and gastrostomy intubation. Patients 1 and 2 achieved minimal manifestation status at week 4 and week 6, respectively, and showed continued improvement on MG‐ADL and MGC scores through weeks 55 and 43, respectively, with eculizumab. The third patient showed a partial response at week 10, followed by a slight decline in his MG‐ADL score, but noted a slow but an incomplete improvement afterward on MG‐ADL and MGC scores, possibly due to delayed eculizumab infusion. Discussion Eculizumab may play a role in the treatment of patients with MGFA class V, refractory gMG. Larger studies are required to provide further evidence.

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Rituximab in refractory myasthenia gravis: Extended prospective study results

Abstract: Sustained clinical improvement was associated with rituximab after 1 cycle, with prolonged time to relapse and reduction in steroid dosage. Muscle Nerve 58: 453-456, 2018.

Cited by 56 publications

References 19 publications

Low‐dose rituximab every 6 months for the treatment of acetylcholine receptor–positive refractory generalized myasthenia gravis

Low‐dose rituximab every 6 months for the treatment of acetylcholine receptor–positive refractory generalized myasthenia gravis

Rituximab in AChR subtype of myasthenia gravis: systematic review

The use of eculizumab in ventilator‐dependent myasthenia gravis patients

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