Diagnosis and Management of Myasthenia Gravis

医学 胸腺切除术 重症肌无力 强的松 硫唑嘌呤 美罗华 伊库利珠单抗 临床试验 他克莫司 内科学 抗体 免疫学 疾病 移植 补体系统
作者
Michael K. Hehir,Yuebing Li
出处
期刊:Continuum [Lippincott Williams & Wilkins]
卷期号:28 (6): 1615-1642 被引量:30
标识
DOI:10.1212/con.0000000000001161
摘要

PURPOSE OF REVIEW: This article reviews updated diagnostic procedures and currently available treatment modalities for myasthenia gravis (MG). RECENT FINDINGS: Patients with MG can be classified based on antibody status and their clinical presentation; treatment responses may differ based on disease subtypes. Improved diagnostic methods and recognition of new antigenic targets such as lipoprotein-related protein 4 have led to improved diagnostic efficiencies. Corticosteroids remain the first-line immunotherapy, but there is a trend toward minimizing their use at high doses and for long durations. Oral immunosuppressants such as mycophenolate mofetil, azathioprine, and tacrolimus remain useful. An international, multicenter randomized trial comparing thymectomy plus prednisone with prednisone alone demonstrated that thymectomy improves clinical outcomes in selected patients with nonthymomatous MG. Eculizumab, efgartigimod, and ravulizumab have recently been approved by the US Food and Drug Administration (FDA) for adult patients with generalized MG who are acetylcholine receptor-antibody positive. These drugs take advantage of novel mechanisms of action and expand treatment options for patients with MG. Data on rituximab suggest that it can be a good option, especially for patients with MG who are positive for antibodies against muscle-specific tyrosine kinase (MuSK). The number of clinical trials and drugs in development for MG is steadily increasing. SUMMARY: The diagnosis of MG can generally be made from the patient's history, a neurologic examination, and laboratory and electrodiagnostic testing. Carefully selected treatment improves outcomes in MG. Additional treatment options for MG will likely be available in the near future.
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