Development of generalized disease at 2 years in patients with ocular myrasthenia gravis
Development of generalized disease at 2 years in patients with ocular myrasthenia gravis
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DOI：
10.1001/archneur.60.2.243
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发表时间：
2003-02-01
影响因子：
--
通讯作者：
Homel, P
中科院分区：
文献类型：
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作者：
Kupersmith, MJ;Latkany, R;Homel, P
背景：超过50%的眼部重症肌无力患者会发展成全身性重症肌无力，通常在2年内。眼肌无力的最佳治疗，包括皮质类固醇的使用，仍然存在争议。目的：评价口服糖皮质激素治疗对眼肌无力患者2年内发生全身性重症肌无力的频率、胸腺瘤发生率以及阳性结果所需依德福胺用量的影响。方法：我们回顾了147例眼肌无力患者的数据库。患者接受了乙酰胆碱受体(AChR)抗体水平和胸部CT检查。除非有禁忌证，否则建议复视患者接受泼尼松治疗，剂量为40~60 mg/d，逐渐减少剂量5~6周。大多数人继续每天或隔日服用2.5至10毫克的剂量，以防止复视。未服用泼尼松的患者(未治疗组)接受溴化吡斯的明治疗或不给予药物治疗。结果：依得芬铵的平均有效剂量为3.3 mg(SD，1.6 mg)，2.6 mg(SD，1.1 mg)，用于治疗上睑下垂。胸腺瘤1例(0.7%)。58例接受治疗的患者中有4例在2年内发展为全身性重症肌无力，36例未治疗的患者中有13例在2年内发展为全身性重症肌无力。与未治疗组相比，治疗组发生全身性疾病的优势比(OR)为0.13(95%可信区间为0.04~0.45)。AChR抗体水平不能预测2年后全身性重症肌无力的发展，但AChR抗体水平异常的患者风险更大(OR，6.33；95%CI，1.71-23.42)。Logistic回归分析显示，年龄、AChR抗体水平异常和泼尼松治疗仅对AChR抗体水平异常(OR，7.03；95%CI，1.35~36.64)和治疗(OR，0.06；95%CI，0.01~0.30)有统计学意义。结论：2年后，泼尼松治疗可将全身性重症肌无力的发生率降至7%，而未接受强的松治疗的患者的发生率为36%。胸腺瘤，虽然不常见，但发生在眼肌无力重症。诊断重症眼肌无力只需要少量的依地芬胺。
Background: Generalized myasthenia gravis will develop in more than 50% of patients who present with ocular myasthenia gravis, typically within 2 years. The optimal treatment of ocular myasthenia gravis, including the use of corticosteroids, remains controversial. In addition, the prevalence of thymoma and the optimal performance of the edrophonium chloride test for ocular myasthenia remain unknown.Objective: To assess the effect of oral corticosteroid therapy on the frequency of development of generalized myasthenia gravis within 2 years, the incidence of thymoma, and the amount of edrophonium needed for a positive test result in patients with ocular myasthenia gravis.Methods: We reviewed an ocular myasthenia gravis database of 147 patients. Patients underwent measurement of acetylcholine receptor (AChR) antibody levels and chest computed tomography. Unless contraindicated, patients with diplopia were recommended for therapy with prednisone, up to 40 to 60 mg/d, with the dosage tapered for 5 to 6 weeks. Most continued to receive daily or alternate-day doses of 2.5 to 10 mg to prevent diplopia. Patients not given prednisone (untreated group) received pyridostigmine bromide or no medication. After the diagnosis, we documented the signs and symptoms of ocular and generalized myasthenia gravis and performed 2-year follow-up in 94 patients.Results: The mean dose of edrophonium chloride to give a positive response was 3.3 mg (SD, 1.6 mg) for ptosis and 2.6 mg (SD, 1.1 mg) for ocular motor dysfunction. Thymoma occurred in 1 patient (0.7%). Generalized myasthenia gravis developed within 2 years in 4 of 58 treated and 13 of 36 untreated patients. The odds ratio (OR) for development of generalized disease in the treated group was 0.13 (95% confidence interval [CI], 0.04-0.45) compared with the untreated group. The AChR antibody level was not predictive of development of generalized myasthenia gravis at 2 years, but the risk was greater in patients with abnormal AChR antibody levels (OR, 6.33; 95% CI, 1.71-23.42). Logistic regression that included age, abnormal AChR antibody level, and prednisone therapy yielded significance only for abnormal AChR antibody level (OR, 7.03; 95% CI, 1.35-36.64) and treatment (OR, 0.06; 95% CI, 0.01-0.30).Conclusions: At 2 years, prednisone treatment appears to reduce the incidence of generalized myasthenia gravis to 7% in contrast to 36% of patients who did not receive prednisone. Thymoma, although uncommon, occurs in ocular myasthenia gravis. Only small amounts of edrophonium are needed to diagnose ocular myasthenia gravis.