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The aim of this retrospective case series study was to evaluate the response and durability of rituximab in patients with new-onset acetylcholine receptor positive (AChR +) generalized myasthenia gravis (MG). Patients were initiated with low-dose rituximab treatment within 3.5 months of onset without concomitant oral immunosuppressants. Seventeen patients (89%) remained relapse-free with a mean follow-up of 51.3 months. Clinical improvement was observed in parallel with the maintenance of low-dose corticosteroids or the complete discontinuation of corticosteroids. Long-term depletion of B cells with low-dose rituximab treatment has shown favorable efficacy and tolerance in reducing disease activity for AChR+ generalized MG., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Neuromyelitis optical (NMO) and neuromyelitis optical spectrum disorder (NMOSD) are inflammatory autoimmune demyelination diseases affecting the central nervous system. We investigated the efficiency of low-dose rituximab treatment in 31 Chinese patients with NMO/NMOSD across a median period of 2.29 ± 0.97 years and azathioprine combined with corticosteroid treatment in 34 Chinese patients with NMO/NMOSD across a median period of 2.61 ± 0.94 years. Among the rituximab-treated patients, the mean Expanded Disability Status Scale (EDSS) was 5.62 ± 1.35 before treatment and 4.48 ± 0.78 at last follow-up, and the mean annualized relapse rate (ARR) was 1.39 ± 0.42 before treatment and 0.03 ± 0.13 at last follow-up. Among the azathioprine-treated patients, the mean EDSS was 5.63 ± 1.29 before treatment and 5.05 ± 1.00 at last follow-up, and the mean ARR was 1.28 ± 0.34 before treatment and 0.49 ± 0.21 at last follow-up. In this study, we showed that using low-dosage rituximab could benefit Chinese patients with NMO by reducing the new occurrence of relapses dramatically. Compared with the azathioprine-treated patients, we concluded that rituximab is more effective in preventing NMO relapse and could improve the symptoms.

Aims: To compare long‐term efficacy and safety of immunotherapeutic strategies as maintenance to prevent disease relapses of generalized myasthenia gravis (MG) in real‐world settings. Methods: This is a retrospective cohort study on generalized MG conducted in seven major neurological centers across China. Eligible participants were patients with generalized MG who were under minimal manifestation status or better. Main outcome measures were probability of patients free of relapses and causes of drug discontinuation. Results: Among 1064 patients enrolled, the median (interquartile range) age was 50.3 (37.0‐62.5) years and 641 (60.2%) were women. Disease relapse was significantly lower for rituximab (6.1%) compared with all the other monotherapies (hazard ratio [HR] = 0.18, 95% confidence interval [CI] 0.06 to 0.56, P =.0030). As combination therapies, tacrolimus in combination with corticosteroids reduced risk of disease relapses compared with azathioprine with corticosteroids (HR = 0.45, 95% CI 0.25 to 0.81, P =.0077) or mycophenolate mofetil with corticosteroids (HR = 0.32, 95% CI 0.15 to 0.67, P =.0020). Otherwise, lower‐dose corticosteroids or azathioprine as monotherapy significantly increased risk of disease relapses (HR = 2.78, 95% CI 1.94 to 3.99, P <.0001; HR = 2.14, 95% CI 1.42 to 3.23, P =.0003, respectively). The proportion of discontinuation was lowest in patients with rituximab (20.4%) as monotherapy and tacrolimus with corticosteroids (23.6%). Overall, combination treatment of immunosuppressants with corticosteroids had a lower rate of discontinuation compared with corresponding monotherapy (HR = 0.51, 95% CI 0.36 to 0.71, P <.0001). Conclusions: Rituximab as monotherapy and tacrolimus with corticosteroids displayed better clinical efficacy as well as drug maintenance to prevent disease relapses in patients with generalized MG. [ABSTRACT FROM AUTHOR]

• Rituximab reduces the relapse frequency in most patients with MOG antibody disease. • There are no differences between rituximab and other immunotherapies in MOG antibody disease. • Rituximab appears less effective for MOG antibody disease than for MS and AQP4-IgG NMOSD. • There was no significant difference between China patients and non- China patients with MOG antibody disease for the effects of rituximab versus other immunotherapies. • A large multicenter randomized controlled clinical trial to thoroughly characterize the efficacy of rituximab for MOG antibody disease is necessary. Myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOG-AD) is now recognised as a nosological entity with specific clinical and paraclinical features to aid early diagnosis. Rituximab (RTX) is a chimeric monoclonal antibody directed against CD20 epitope expressed on pre-B and mature B cells and is used to treat B-cell-derived lymphoid neoplasms and antibody-mediated autoimmune diseases. In this review, we performed a meta-analysis to evaluate RTX efficacy and assessed the treatment efficacies based on relapse rates. This study was conducted according to the PRISMA (Preferred Reporting Items for Systemic review and Meta-Analysis) statement. We searched for publications on the PubMed, Embase, Cochrane Library, clinical trials up to December 2020. We compiled 5 studies, Meta-analysis forest plots was conducted for the ARR ratio change pre and post-treatment between rituximab and other disease modifying drugs. A sensitivity analysis was performed with mean difference (MD) of the efficacy of RTX versus other immunotherapies and subgroup analysis was also performed based on site of study. A meta-analysis of 5 studies with 239 participants was conducted. Patients have received rituximab were recorded in 82 of 239 (34.31%). The mean difference of ARR ratio of rituximab therapy versus other immunotherapies was 0.16 (95%CI, −0.15 to 0.47). No studies found to significantly affect heterogeneity. No major differences occurred in 9.2% of China patients (95% CI: -0.20—1.86; I2=0%) and 90.8% of non- China patients (95% CI: -0.24–0.42; I2=0%). Meanwhile there was no significant subgroup difference (p = 0.18) between them. RTX reduces the relapse frequency in most patients with MOG antibody disease, but there is no differences between rituximab and other immunotherapies in MOG antibody disease. Future a large multicenter randomized controlled clinical trial to thoroughly characterize the efficacy of rituximab for MOG antibody disease is necessary. [ABSTRACT FROM AUTHOR]