Kaminski Henry J, Denk Jordan
Department of Neurology and Rehabilitation Medicine, George Washington University, Washington, DC, United States.
Front Neurol. 2022 Apr 25;13:886625. doi: 10.3389/fneur.2022.886625. eCollection 2022.
Chronic, high-dose, oral prednisone has been the mainstay of myasthenia gravis treatment for decades and has proven to be highly beneficial in many, toxic in some way to all, and not effective in a significant minority. No patient characteristics or biomarkers are predictive of treatment response leading to many patients suffering adverse effects with no benefit. Presently, measurements of treatment response, whether taken from clinician or patient perspective, are appreciated to be limited by lack of good correlation, which then complicates correlation to biological measures. Treatment response may be limited because disease mechanisms are not influenced by corticosteroids, limits on dosage because of adverse effects, or individual differences in corticosteroids. This review evaluates potential mechanisms that underlie lack of response to glucocorticoids in patients with myasthenia gravis.
几十年来,长期、大剂量口服泼尼松一直是重症肌无力治疗的主要手段,已被证明对许多患者非常有益,对所有患者都有某种程度的毒性,而对相当一部分患者则无效。没有患者特征或生物标志物能够预测治疗反应,导致许多患者遭受不良反应却没有益处。目前,无论是从临床医生还是患者的角度衡量治疗反应,都因缺乏良好的相关性而受到限制,这进而使与生物学指标的关联变得复杂。治疗反应可能受到限制,原因可能是疾病机制不受皮质类固醇影响、因不良反应导致剂量受限,或者是皮质类固醇存在个体差异。本综述评估了重症肌无力患者对糖皮质激素无反应背后的潜在机制。