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

Author:

Zhang Jieni,Chen Yin,Chen Jiaxin,Huang Xin,Wang Haiyan,Li Yan,Liu Weibin,Feng HuiyuORCID

Abstract

Abstract 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.

Funder

the Project of Guangzhou Science Technology and Innovation Commission

Natural Science Foundation of Jilin Province

National Key Clinical Specialty Discipline Construction Program of China

International Foundation for Functional Gastrointestinal Disorders

Guangdong Provincial Engineering Center For Major Neurological Disease Treatment

Guangdong Provincial Translational Medicine Innovation Platform for Diagnosis and Treatment of Major Neurological Disease

Guangdong Provincial Clinical Research Center for Neurological Diseases

Publisher

Springer Science and Business Media LLC

Subject

Psychiatry and Mental health,Neurology (clinical),Dermatology,General Medicine

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