Department of Ophthalmology, University of Alabama Birmingham, Birmingham, Alabama.
Department of Neurology, Coimbra University Hospital Center, Praceta Professor Mota Pinto, Coimbra, Portugal.
Ophthalmology. 2015 Jul;122(7):1517-21. doi: 10.1016/j.ophtha.2015.03.010. Epub 2015 Apr 17.
To calculate the rate and timing of conversion from ocular myasthenia gravis to generalized myasthenia gravis.
Retrospective multicenter analysis.
Patients included in the study were diagnosed with ocular myasthenia gravis without the presence of generalized disease at onset.
We conducted a retrospective multicenter analysis. We reviewed charts of 158 patients who met diagnostic criteria for ocular myasthenia gravis. Patients were divided into 2 subgroups: an immunosuppressant treatment group and a nonimmunosuppressant treatment group. Timing of conversion to generalized disease and duration of follow-up also was evaluated. Additional data such as clinical symptoms at presentation, laboratory test results, and chest imaging results also were recorded.
Conversion rates to generalized myasthenia at 2 years, effect of immunosuppression on conversion, and timing of conversion.
The 158-patient cohort included 76 patients who received immunosuppressant therapy; the remaining 82 patients did not. The overall conversion rate to generalized disease was 20.9%. At 2 years, generalized myasthenia developed in 8 of 76 patients in the treated group and in 15 of 82 patients in the nonimmunotherapy group (odds ratio, 0.52; 95% confidence interval, 0.20-1.32). Median time for conversion to generalized disease was 20 months in the nonimmunosuppressant group and 24 months in the immunosuppressant group. Conversion occurred after 2 years of symptom onset in 30% of patients.
Conversion rates from ocular to generalized myasthenia gravis may be lower than previously reported both in immunosuppressed and nonimmunosuppressed patients. A subset of patients may continue to convert to generalized disease beyond 2 years from onset of symptoms, and close monitoring should be continued.
计算眼肌型重症肌无力向全身型重症肌无力转化的速率和时间。
回顾性多中心分析。
本研究纳入的患者在发病时即被诊断为眼肌型重症肌无力,且不存在全身型疾病。
我们进行了一项回顾性多中心分析。我们回顾了符合眼肌型重症肌无力诊断标准的 158 例患者的病历。患者被分为 2 个亚组:免疫抑制剂治疗组和非免疫抑制剂治疗组。我们评估了向全身型疾病转化的时间和随访时间,还评估了其他数据,如发病时的临床症状、实验室检查结果和胸部影像学结果。
2 年内向全身型重症肌无力的转化率、免疫抑制治疗对转化的影响以及转化时间。
这 158 例患者队列包括 76 例接受免疫抑制剂治疗的患者;其余 82 例患者未接受免疫抑制剂治疗。总体向全身型疾病的转化率为 20.9%。在治疗组中,2 年内有 8 例患者发展为全身型重症肌无力,而非免疫治疗组中有 15 例患者(比值比,0.52;95%置信区间,0.20-1.32)。非免疫抑制剂组向全身型疾病转化的中位时间为 20 个月,免疫抑制剂组为 24 个月。30%的患者在发病 2 年后出现转化。
与既往报道相比,免疫抑制和非免疫抑制患者的眼肌型重症肌无力向全身型重症肌无力的转化率可能较低。有一部分患者可能在症状出现后 2 年继续向全身型疾病转化,因此应继续密切监测。