Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Front Immunol. 2023 May 17;14:1177249. doi: 10.3389/fimmu.2023.1177249. eCollection 2023.
To describe the clinical predictors and immune-related factors for exacerbation in adults with well-controlled generalized myasthenia gravis (GMG).
We conducted a retrospective analysis of 585 adults with well-controlled GMG from our institution to explore the risk factors for exacerbation. Furthermore, propensity score matching (PSM) was used to compare the proportions of lymphocyte subsets, and the levels of immunoglobulin, complement, and anti-acetylcholine receptor antibody (AChR-ab) in the peripheral blood of 111 patients with exacerbations and 72 patients without exacerbations.
A total of 404 patients (69.1%) experienced at least one exacerbation, and the median (interquartile range) time to the first exacerbation was 1.5 years (0.8-3.1 years). Multivariable Cox regression analysis showed that age at onset, disease duration before enrollment, Myasthenia Gravis Foundation of America classification (MGFA) class III vs. class II, MGFA class IV-V vs. class II, AChR-ab levels, anti-muscle specific kinase antibody levels, thymus hyperplasia, prednisone plus immunosuppressants vs. prednisone treatment, and thymectomy were independent predictors for exacerbations [hazard ratio (HR) = 1.011, 1.031, 1.580, 1.429, 2.007, 2.033, 1.461, 0.798, and 0.651, respectively]. Propensity-matched analysis compared 51 patient pairs. After PSM, the peripheral blood proportions of CD3CD19 B cells, ratios of CD3CD4/CD3CD8 T cells, and AChR-ab levels were significantly increased, and the peripheral blood proportions of CD3CD8 T and CD4CD25CD127 regulatory T cells (Tregs) were significantly lower in patients with exacerbation than in those without exacerbation (all < 0.05).
Myasthenia gravis (MG) exacerbations were more frequent in those patients with older onset age, longer disease duration, more severe MGFA classification, positive AChR-ab, and lack of combined immunotherapy or thymectomy treatment. On the other hand, CD3CD19 B cells, CD3CD8 T cells, Tregs, and AChR-ab in peripheral blood may be involved in the course of GMG exacerbation.
描述成人重症肌无力（GMG）病情控制良好患者加重的临床预测因素和免疫相关因素。
我们对我院 585 例病情控制良好的成人 GMG 患者进行了回顾性分析，以探讨加重的危险因素。此外，采用倾向评分匹配（PSM）比较了 111 例加重患者和 72 例无加重患者外周血淋巴细胞亚群比例、免疫球蛋白、补体和乙酰胆碱受体抗体（AChR-ab）水平。
共有 404 例（69.1%）患者至少发生 1 次加重，首次加重的中位（四分位距）时间为 1.5 年（0.8-3.1 年）。多变量 Cox 回归分析显示，发病年龄、入组前病程、美国重症肌无力基金会分类（MGFA）III 级与 II 级、MGFA IV-V 级与 II 级、AChR-ab 水平、抗肌肉特异性激酶抗体水平、胸腺增生、泼尼松加免疫抑制剂与泼尼松治疗、胸腺切除术是加重的独立预测因素[风险比（HR）分别为 1.011、1.031、1.580、1.429、2.007、2.033、1.461、0.798 和 0.651]。倾向性匹配分析比较了 51 对患者。PSM 后，加重患者外周血 CD3CD19 B 细胞比例、CD3CD4/CD3CD8 T 细胞比值及 AChR-ab 水平明显升高，外周血 CD3CD8 T 及 CD4CD25CD127 调节性 T 细胞（Tregs）比例明显降低（均<0.05）。
起病年龄较大、病程较长、MGFA 分级较重、AChR-ab 阳性、未行联合免疫治疗或胸腺切除术的患者，MG 加重更为频繁。另一方面，外周血 CD3CD19 B 细胞、CD3CD8 T 细胞、Tregs 和 AChR-ab 可能参与了 GMG 加重的过程。
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