Narita Tomoko, Nakane Shunya, Nagaishi Akiko, Minami Naoya, Niino Masaaki, Kawaguchi Naoki, Murai Hiroyuki, Kira Jun-Ichi, Shimizu Jun, Iwasa Kazuo, Yoshikawa Hiroaki, Hatanaka Yuki, Sonoo Masahiro, Shimizu Yuko, Matsuo Hidenori
Department of Neurology, National Hospital Organization, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
Department of Neurology, Nippon Medical School, Tokyo, Japan.
Ther Adv Neurol Disord. 2023 Apr 4;16:17562864231163819. doi: 10.1177/17562864231163819. eCollection 2023.
Treatment for ocular myasthenia gravis (OMG) has not yet been well established. Few reports have been published on the clinical practice and outcomes of OMG.
We investigated treatment of OMG and its outcomes in Japan.We investigated treatment of OMG and its outcomes in Japan.
We performed a retrospective cross-sectional survey of OMG patients from eight hospitals in Japan.
Clinical information, including sex, age at onset, initial symptoms, autoantibodies, clinical course, treatment history, complications, and outcomes, was obtained. In addition, we recorded the total number of patients with MG and OMG separately.
In total, 135 patients with OMG (67 men, 68 women) were included. Treatment of OMG was not simple and involved various immunotherapeutic strategies. Eight patients went into remission spontaneously without immunotherapy. A total of 117 patients showed improvements after treatment, whereas 10 patients showed refractory responses to treatment. Overall outcomes were good; however, symptoms persisted in 60.7% of patients even after treatment. Among 90 patients who received immunotherapy, only two showed a refractory response. Meanwhile, for 45 patients who did not receive immunotherapy, 8 were refractory. Thus, the rate of refractory disease in the group with immunotherapy was significantly lower ( = 0.001, u-test) than in the group without immunotherapy. The proportion of generalized MG patients among all MG cases was low in medical centers where immunotherapy for OMG was frequently performed.
Although the overall prognosis for patients with OMG was good, symptoms remained in more than half of the patients. Immunotherapy, including corticosteroids, may be beneficial for patients with OMG.
Patients with ocular myasthenia gravis (OMG) have only eye symptoms for more than 2 years. Whether this condition is an initial stage of the disease before eventually progressing to generalized myasthenia gravis (gMG) is still uncertain. Different from gMG, OMG is not life-threatening. But eye symptoms often cause troublesome problems in life. Doctors have treated OMG patients similarly to patients with gMG. There is no standard clinical practice for OMG. In this study, we examined how patients with OMG were treated at eight different specialist centers in Japan. In 135 patients with OMG, 8 patients became symptom free without treatment, 117 patients showed improvements after treatment, whereas 10 patients did not get well. Overall outcomes were good, but symptoms remained in 60.7% of patients even after treatment. Among 90 patients who received one or more immunotherapies, only 2 did not get well. Meanwhile, for 45 patients who did not receive immunotherapy, 8 remained ill. We found that treatment of OMG was not simple and often needed multiple immunotherapies. Administering immunotherapy, including corticosteroids, may be beneficial for patients with OMG.
眼肌型重症肌无力（OMG）的治疗方法尚未完全确立。关于OMG的临床实践和治疗结果的报道较少。
我们调查了日本OMG的治疗方法及其结果。
我们对日本八家医院的OMG患者进行了回顾性横断面调查。
获取了包括性别、发病年龄、初始症状、自身抗体、临床病程、治疗史、并发症和治疗结果等临床信息。此外，我们分别记录了MG和OMG患者的总数。
总共纳入了135例OMG患者（67例男性，68例女性）。OMG的治疗并不简单，涉及多种免疫治疗策略。8例患者未经免疫治疗自发缓解。共有117例患者治疗后症状改善，而10例患者对治疗反应不佳。总体治疗结果良好；然而，即使在治疗后仍有60.7%的患者症状持续存在。在90例接受免疫治疗的患者中，只有2例反应不佳。同时，在45例未接受免疫治疗的患者中，有8例反应不佳。因此，免疫治疗组的难治性疾病发生率显著低于（u检验，P = 0.001）未接受免疫治疗组。在频繁进行OMG免疫治疗的医疗中心，所有MG病例中全身型MG患者的比例较低。
尽管OMG患者的总体预后良好，但仍有超过一半的患者症状持续存在。包括皮质类固醇在内的免疫治疗可能对OMG患者有益。
眼肌型重症肌无力（OMG）患者在两年多时间里仅有眼部症状。这种情况是否是疾病最终发展为全身型重症肌无力（gMG）的初始阶段仍不确定。与gMG不同，OMG不会危及生命。但眼部症状在生活中常常会带来麻烦。医生对OMG患者的治疗方式与gMG患者类似。OMG没有标准的临床治疗方法。在本研究中，我们调查了日本八个不同专科中心对OMG患者的治疗情况。在135例OMG患者中，8例未经治疗症状消失，117例治疗后症状改善，而10例患者病情未好转。总体治疗结果良好，但即使在治疗后仍有60.7%的患者症状持续存在。在90例接受一种或多种免疫治疗的患者中，只有2例病情未好转。同时，在45例未接受免疫治疗的患者中，有8例仍未康复。我们发现OMG的治疗并不简单，通常需要多种免疫治疗。给予包括皮质类固醇在内的免疫治疗可能对OMG患者有益。
Zotero 插件
