Rituximab for myasthenia gravis Three case reports and review of the literature
ABSTRACT In generalized myasthenia gravis (MG), a wide array of immunosuppressive and immunomodulating treatments is being used in clinical practice, but most drugs lack evidence from randomized controlled trials supporting their use. Furthermore, many patients develop serious side effects or do not respond sufficiently to these drugs. We report three patients with generalized MG who were treated with rituximab, a monoclonal antibody against CD20+ cells that causes prolonged B cell depletion. In all three patients, treatment with rituximab led to a sustained clinical improvement and discontinuation or reduction of prednisolone and other drugs. Rituximab was well tolerated. Therapy with rituximab was guided by the total count of peripheral B lymphocytes. Reviewing the anecdotal literature on rituximab for MG, we conclude that preliminary data on the efficacy and safety of rituximab are encouraging and that further studies in MG seem warranted.
SourceAvailable from: onlinelibrary.wiley.com[Show abstract] [Hide abstract]
ABSTRACT: This article provides a thorough overview of the current advances in diagnosis and therapy of myasthenia gravis (MG). Nowadays the term 'myasthenia gravis' includes heterogeneous autoimmune diseases with a postsynaptic defect of neuromuscular transmission as the common feature. Myasthenia gravis should be classified according to the antibody specificity (acetylcholine, MuSK, LRP4, seronegative), thymus histology (thymitis, thymoma, atrophy), age at onset (in children; < or > 40 years), and type of course (ocular or generalized). With optimal treatment, the prognosis is good in terms of daily functions, quality of life and survival. Symptomatic treatment with acetylcholine esterase inhibition is usually combined with immunosuppression. Azathioprine remains still the first choice for long-term immunosuppressive therapy. Alternative immunosuppressive options to azathioprine include ciclosporin, cyclophosphamide, methotrexate, mycophenolate mofetil, and tacrolimus. Rituximab is a promising new drug for severe generalized MG. Emerging therapy options include belimumab, eculizumab, and the granulocyte macrophage-colony-stimulating factor. One pilot study on etanercept has given disappointing results. For decades thymectomy has been performed in younger adults to improve non-paraneoplastic MG. However, controlled prospective studies on the suspected benefit of this surgical procedure are still lacking. In acute exacerbations including myasthenic crisis, intravenous immunoglobulin, plasmapheresis, and immunoadsorption are similarly effective.Clinical & Experimental Immunology 10/2013; DOI:10.1111/cei.12217 · 3.28 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: Myasthenia gravis is an autoimmune disorder of the neuromuscular junction caused by circulating antibodies specif ic for the post-synaptic acetylcholine receptor or, in a minority of cases, for the muscle-specific tyrosine-kinase and the low-density lipoprotein receptor-related protein 4. A wide range of symptomatic and immunosuppressive treatments is currently available for MG patients with variable outcome. However, most immunosuppressive treatments are characterized by delayed onset of action and in some cases are not sufficient to induce stable remission of the disease. Rituximab (RTX) is a chimaeric monoclonal antibody specific for the CD20 B-cell surface antigen. Recent studies have provided evidence that RTX may be an effective treatment for patients with myasthenia gravis (MG) who are refractory to standardized immunosuppressive therapy. We performed a systematic review and a meta-analysis of the efficacy and safety of RTX in myasthenia gravis considering the potential predictive factors related to patients' response to RTX in this disease.Journal of Neurology 10/2014; DOI:10.1007/s00415-014-7532-3 · 3.84 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: Myasthenia gravis (MG) is the most common disorder of neuromuscular transmission and is a prototypical autoimmune disorder. Most patients with MG are successfully treated with acetylcholinesterase inhibitors, corticosteroids, and/or steroid sparing agents such as azathioprine and mycophenolate mofetil. There is a small subset of patients, however, with treatment-refractory disease. In these cases, medications such as rituximab, high-dose cyclophosphamide, and eculizumab may be used. Thymectomy (in some cases repeat thymectomy) is another option in selected patients. Studies evaluating these and other forms of therapy in treatment-refractory MG are reviewed.Journal of Clinical Neuromuscular Disease 06/2014; 15(4):167-78. DOI:10.1097/CND.0000000000000034