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AChRAb and MuSKAb double-seropositive myasthenia gravis: a distinct subtype?

期刊

NEUROLOGICAL SCIENCES
卷 42, 期 3, 页码 863-869

出版社

SPRINGER-VERLAG ITALIA SRL
DOI: 10.1007/s10072-021-05042-3

关键词

Myasthenia gravis; Antibody; Acetylcholine receptor; Muscle-specific tyrosine kinase; Double-seropositive myasthenia gravis; Subtype

资金

  1. Project of Guangzhou Science Technology and Innovation Commission [201707010122]
  2. Guangdong Natural Science Foundation Committee [2017A030313829, 2018A030313449]
  3. National Key R&D Program of China [2017YFC0907700]
  4. Southern China International Cooperation Base for Early Intervention and Functional Rehabilitation of Neurological Diseases [2015B050501003]
  5. Guangdong Provincial Engineering Center For Major Neurological Disease Treatment
  6. Guangdong Provincial Clinical Research Center for Neurological Diseases
  7. Guangdong Provincial Translational Medicine Innovation Platform for Diagnosis and Treatment of Major Neurological Disease

向作者/读者索取更多资源

The study found that DSP-MG in southern China has more severe symptoms and poor prognosis compared to AChR-MG, with no significant differences compared to MuSK-MG. The researchers speculated that DSP-MG may be a subtype of MuSK-MG.
Introduction This study investigated the characteristics of double-seropositive myasthenia gravis (DSP-MG) in southern China for disease subtype classification. Methods A case-control study was carried out in which the characteristics of DSP-MG patients (n = 17) were compared to those of muscle-specific tyrosine kinase antibody-positive (MuSK)-MG and acetylcholine receptor antibody-positive (AChR)-MG patients (n = 8 and 27, respectively). We also performed a literature review of DSP-MG patients. Results Compared to AChR-MG, DSP-MG had greater bulbar dysfunction (47.1% vs 18.6%, P = 0.04), higher incidence of myasthenia crisis (41.2% vs 14.8%, P = 0.04), more severe Myasthenia Gravis Foundation of America classification at maximum worsening, greater autoantibody abnormalities (70.6% vs 33.3%, P = 0.015), greater need for immunosuppressant treatment (58.8% vs 3.7%, P < 0.001), and worse prognosis with less remission (11.8% vs 55.6%, P = 0.001). There were no differences between DSP-MG and MuSK-MG patients. DSP-MG described in published reports was comparable to MuSK-MG. Discussion DSP-MG in southern China may be a subtype of MuSK-MG.

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