The co-existence of myasthenia gravis in patients with myositis: a case series.

The co-existence of myasthenia gravis in patients with myositis: a case series.
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DOI:
10.1016/j.semarthrit.2013.12.005
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发表时间:
2014-06
影响因子:
5
通讯作者:
Mammen AL
Mammen AL
中科院分区:
医学2区
文献类型:
--
作者:
Paik JJ;Corse AM;Mammen AL

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肌炎和重症肌无力(MG)都是自身免疫性疾病,表现为肌肉无力。它们很少同时发生在同一病人身上。由于重症肌无力的治疗不同于肌炎的治疗,所以当患者同时患有这两种疾病时,认识到这一点是很重要的。我们回顾了6例同时患有肌炎和MG的患者,以确定提示肌炎患者可能同时存在MG的临床特征。我们确定了6例皮肌炎或多发性肌炎合并MG的患者。我们回顾了他们的医疗记录,以评估他们的临床表现、实验室结果和电生理特征。根据Bohan和Peter标准以及MG的电生理和/或血清学证实,6例患者均明确为皮肌炎或多发性肌炎。重叠患者中5/6(83%)有球无力,2/6(33%)有上睑下垂,1/6(17%)有复视。5/6(83%)的患者存在疲劳性虚弱。吡哆斯的明治疗改善了5/6(83%)的症状。高剂量类固醇与2/6(33%)患者虚弱加重相关。突出的球症状、上睑下垂、复视和疲劳无力提示肌炎患者MG的可能性。怀疑MG可通过适当的电生理和实验室检查加以证实。在肌炎- mg重叠的患者中,大剂量类固醇可能加重症状,吡多斯的明可能发挥重要的治疗作用。
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%). 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 pryidostigmine may play an important therapeutic role.