期刊
JOURNAL OF NEUROIMMUNOLOGY
卷 371, 期 -, 页码 -出版社
ELSEVIER
DOI: 10.1016/j.jneuroim.2022.577950
关键词
Thymoma; Myasthenia gravis; Immunodeficiency; Immunoglobulin G; Infections
资金
- JSPS KAKENHI [JP20H03592]
Thymoma-associated myasthenia gravis (MG) with immunodeficiency has distinct clinical and immunological features, including severe pneumonia, invasive thymoma, and autoimmune disorders. Compared to patients without immunodeficiency, there were no significant differences in the severity of MG, but lower IgG concentrations and higher mortality rates were observed.
Thymoma with immunodeficiency is sometimes accompanied by myasthenia gravis (MG), but the clinical characteristics have not been elucidated. This study aimed to characterize its clinical and immunological fea-tures. Of the 132 thymoma-associated MG patients, 9 patients presented with immunodeficiency. All suffered from severe pneumonia, and most had invasive thymoma and autoimmune disorders. DRB1*08:03 and DQB1*06:01 alleles were frequently detected. Compared to group without immunodeficiency, they showed no significant differences in the severity of MG, significantly lower IgG concentrations and higher mortality rate. Thymoma-associated MG with immunodeficiency is a distinct subset requiring special attention to prevent infection during the follow-up period.
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