MuSK (Muscle Specific Kinase) Positive Myasthenia: Grave Prognosis or Undue Prejudice?
MuSK (Muscle Specific Kinase) Positive Myasthenia: Grave Prognosis or Undue Prejudice?
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MuSK（肌肉特异性激酶）阳性肌无力：严重预后还是不当偏见？
DOI：
10.4103/aian.aian_302_19
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发表时间：
2020
影响因子：
1.7
通讯作者：
M. Padma Srivastava
中科院分区：
文献类型：
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作者：
P. Samal;V. Goyal;Mamta B. Singh;M. Padma Srivastava
目的：肌肉特异性激酶（MuSK）阳性的肌无力患者通常被认为具有严重的预后。我们提出我们的经验与不同抗体状态的肌无力患者。接下来是对以前的研究和目前对MuSK阳性肌无力的看法的简短论述，重点是相关的偏见。材料与方法：本研究比较了三级中心的23例MuSK阳性肌无力患者、55例乙酰胆碱受体阳性肌无力患者和9例双血清阴性肌无力患者。结果如下：在临床特征、免疫抑制剂治疗反应、长期预后和生活质量方面，我们没有发现任何显著差异。结论：血清抗体阳性不应单独用于指导治疗或预测预后。不适当的负面宣传可能会影响病人的士气。在计划治疗前，除了抗体状态外，还必须考虑临床特征和对治疗的反应。
Objectives: Patients with muscle-specific kinase (MuSK)-positive myasthenia are generally considered to have a grave prognosis. We present our experience of patients with myasthenia with different antibody status. This is followed by a short discourse on previous studies and the current view on MuSK-positive myasthenia, focusing on the associated prejudice. Materials and Methods: This study compares 23 patients with MuSK-positive myasthenia with 55 patients with acetylcholine receptor–positive myasthenia and 9 patients with double-seronegative myasthenia at a tertiary level center. Results: We did not find any significant difference in terms of clinical characteristics, treatment response to immunosuppressants, long-term prognosis, and quality of life. Conclusion: Seropositivity for antibodies should not be used in isolation to guide the management or predict the prognosis. Undue negative prognostication may affect the morale of patient. Clinical features and response to therapy in addition to antibody status must be considered before planning therapy.