A case of Sj?gren′s syndrome combined with myasthenia gravis with literature review
10.3760/cma.j.cn141217-20240430-00147
- VernacularTitle:干燥综合征合并重症肌无力1例及文献复习
- Author:
Xueliang LIU
1
;
Dongyu CHEN
1
;
Fang LI
1
;
Shupeng WU
1
;
Xue LI
1
;
Cong LIU
1
;
Jiatian ZHENG
1
Author Information
1. 泰安市中心医院风湿免疫科，泰安 271000
- Publication Type:Journal Article
- Keywords:
Sj?gren′s syndrome;
Myasthenia gravis;
Autoimmune diseases
- From:
Chinese Journal of Rheumatology
2025;29(8):681-686
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To enhance the understanding of clinical doctors on the concurrence of Sj?gren′s syndrome (SS) and myasthenia gravis (MG), provide reference forclinical diagnosis and treatment.Methods:The medical record of SS、MG patient Taian Central hospital in october 2021 was reviewed and a retrospective analysis was conducted. Based on the key words, the main databases domestic and abroad from Jan 1973 to April 2024. The cases reported in the literature were searched out and merged with our case and analyzed by SPSS 27.0. The measurement data of normal distribution were expressed by mean standard deviation ± s, and the measurement data of abnormal distribution were expressed by M( Q1, Q3). Counting data were expressed as examples and percentages (%) . Results:Twenty-one patients with SS complicated with MG were reviewed. Among them, 19(90.5%) patients were female. The average age was (47.6 ± 3.1) years, and the median time from onset to diagnosis was 24 (11, 78) months. There were 11 patients (52.4%) with myasthenia gravis as the initial presentation. The main clinical manifestations of patients with myasthenia muscle fatigue (80.9%, 17/21), gravis include fatigue ptosis (71.4%, 15/21), diplopia, blurred vision (57.1%, 12/21), dysphagia (33.3%, 7/21), joint pain (33.3%, 7/21), odysarthria, and unclear speech (23.8%, 5/21), hair loss (14.3%, 3/21), and swelling of the excocrine glands(9.5%, 2/21). Eighteen patients (85.7%, 18/21) showed positive anti acetylcholine receptor antibodies. Sixteen patients (76.2%) were positive for ANA, and 14 patients (66.8%) were positive for anti SSA or (and) anti SSB antibodies. Four patients (19.0%) also had other autoimmune diseases (Hashimoto′s thyroiditis, rheumatoid arthritis, optic neuritis). In terms of treatment, cholinesterase inhibitors were the most common treatment measure, applied to 18 patients (85.7%), followed by glucocorticoid therapy, and applied to 14 patients (66.8%). In addition, 7 patients (33.3%) received immunosuppressive agents therapy, 5 patients (23.8%) underwent thymectomy, 2 patients (9.5%) received intravenous human immunoglobulin injection, and 1 patient (4.8%) underwent plasma exchange. All patients showed improvement after treatment.Conclusion:SS and MG are both autoimmune diseases, and their coexistence is rare. Clinicans should be aware of this rare association. Early diagnosis is crucial for the treatment and prognosis of patients,and requiresa comprehensive assessment of clinical symptoms, laboratory tests, and auto-antibody test results.
