Verma Rashmi, Wolfe Gil I, Kupersmith Mark J
Department of Neurology, Mount Sinai Beth Israel, New York, NY, United States of America.
Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, Univ. at Buffalo/ SUNY, Buffalo, NY, United States of America.
J Neurol Sci. 2021 Jan 15;420:117274. doi: 10.1016/j.jns.2020.117274. Epub 2020 Dec 17.
Controversy persists on the best treatment to control ocular myasthenia gravis (OMG) and reduce conversion to generalized myasthenia gravis (GMG). We hypothesized that low dose prednisone could accomplish both in a cohort of OMG patients followed after three years.
We reviewed the records of 168 patients who presented with OMG. Our study included 103 of the OMG patients who met inclusion criteria, requiring follow up for a minimum of 3 years without disease generalization. Low dose prednisone was defined as ≤7.5 mg per day. The main outcome was having single vision without ptosis blocking vision, measured by binocular single vision (BSV) and upper lid position. We also analyzed late progression to GMG.
Of 87 patients treated with prednisone, chronic low dose prednisone alone restored BSV in 47 patients (46% of all patients) without GMG. Pyridostigmine monotherapy restored BSV in 11/14 patients (11% of all patients). Other immunomodulatory therapy (OIT) was needed in 38 patients (37%). Medical therapy maintained BSV at last evaluation (mean follow up 8.2 ± 5.0 years) in 93 patients (90%). GMG developed in 10 patients (10%) during the follow-up period.
In OMG patients who do not generalize before 3 years, chronic long term prednisone at lower doses is moderately effective in maintaining optimum BSV. However, OIT are commonly required in these patients. In these OMG patients receiving prednisone and/or OIT, conversion to GMG after three years of disease is uncommon.
关于控制眼肌型重症肌无力（OMG）以及减少其转化为全身型重症肌无力（GMG）的最佳治疗方法仍存在争议。我们假设低剂量泼尼松能够在一组随访三年的OMG患者中实现这两个目标。
我们回顾了168例OMG患者的病历。我们的研究纳入了103例符合纳入标准的OMG患者，这些患者需要至少随访3年且病情未泛化。低剂量泼尼松定义为每天≤7.5毫克。主要结局是通过双眼单视（BSV）和上睑位置测量，达到无睑下垂遮挡视力的单视。我们还分析了向GMG的晚期进展情况。
在87例接受泼尼松治疗的患者中，单纯慢性低剂量泼尼松使47例患者（占所有患者的46%）恢复了BSV且未发生GMG。吡啶斯的明单药治疗使11/14例患者（占所有患者的11%）恢复了BSV。38例患者（37%）需要其他免疫调节治疗（OIT）。在最后一次评估时（平均随访8.2±5.0年），93例患者（90%）通过药物治疗维持了BSV。随访期间有10例患者（10%）发生了GMG。
在3年内病情未泛化的OMG患者中，低剂量的慢性长期泼尼松在维持最佳BSV方面有中等效果。然而，这些患者通常需要OIT。在这些接受泼尼松和/或OIT治疗的OMG患者中，患病三年后转化为GMG的情况并不常见。
