Paik Julie J, Corse Andrea M, Mammen Andrew L
Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD.
Semin Arthritis Rheum. 2014 Jun;43(6):792-6. doi: 10.1016/j.semarthrit.2013.12.005. Epub 2013 Dec 18.
Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients.
We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features.
All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%) patients. High-dose steroids were associated with worsening weakness in 2/6 (33%) patients.
Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis-MG overlap, high-dose steroids may exacerbate symptoms and pyridostigmine may play an important therapeutic role.
肌炎和重症肌无力(MG)均为表现为肌无力的自身免疫性疾病。它们极少在同一患者中同时出现。由于重症肌无力的治疗方法与肌炎不同,因此识别患者同时患有这两种疾病很重要。我们回顾了6例同时患有肌炎和MG的患者病例,以确定提示肌炎患者合并MG可能性的临床特征。
我们确定了6例患有皮肌炎或多发性肌炎以及MG的患者。我们回顾了他们的病历,以评估其临床表现、实验室检查结果和电生理特征。
根据Bohan和Peter的标准,所有6例患者均确诊为皮肌炎或多发性肌炎,同时经电生理和/或血清学证实患有MG。在重叠患者中,5/6(83%)有延髓肌无力,2/6(33%)有上睑下垂,1/6(17%)有复视。5/6(83%)的患者存在易疲劳性肌无力。6例患者中有5例(83%)使用吡啶斯的明治疗后症状改善。2/6(33%)的患者使用高剂量类固醇后肌无力加重。
突出的延髓症状、上睑下垂、复视和易疲劳性肌无力提示肌炎患者可能合并MG。通过适当的电生理和实验室检查可确诊MG。在肌炎-MG重叠患者中,高剂量类固醇可能会加重症状,而吡啶斯的明可能发挥重要的治疗作用。