眼肌型重症肌无力胸腺切除术-预后和风险因素分析。
Thymectomy in ocular myasthenia gravis-prognosis and risk factors analysis.
机构信息
Department of Cardiothoracic Surgery, Tianjin Medical University General Hospital, No.154, Anshan Road, Tianjin, China.
出版信息
Orphanet J Rare Dis. 2022 Aug 9;17(1):309. doi: 10.1186/s13023-022-02454-y.
BACKGROUND
Several retrospective studies have identified risk factors associated with ocular myasthenia gravis (OMG) generalization in non-surgical patients. However, the outcomes of OMG after thymectomy have not been investigated fully. This study aimed to explore the clinical predictors of post-thymectomy OMG prognosis.
METHODS
We performed a retrospective review of OMG patients who underwent thymectomy at our institution from January 2012 to December 2021. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between clinical features and prognosis. The main outcome measures were OMG conversion, complete stable remission (CSR), and clinical improvement.
RESULTS
Fifty-eight patients were identified for conversion analysis. Thirteen (22.4%) developed generalized myasthenia gravis (GMG) at a median time of 12.7 (3-37.3) months from symptom onset. Repetitive nerve stimulation (RNS)-positivity was associated with increased risk of conversion to GMG (P = 0.002). Patients with histotype B2/B3 thymoma showed a higher risk of conversion (P = 0.002) than did patients with hyperplasia and AB/B1 thymoma. Fifty-two patients fulfilled the criteria for CSR and improvement. Sixteen (30.8%) achieved CSR at a median time of 28.7 (15-54) months after thymectomy. Fifteen (28.8%) showed clinical improvement at last follow up. Patients who achieved CSR showed a younger age of onset (P = 0.022), lower percentage of acetylcholine receptor antibody-seropositivity (P = 0.029). Histologically, patients with thymic hyperplasia and stage I thymoma showed a higher chance of CSR (P = 0.010) than did patients with stage II/III thymoma. Multivariate analysis revealed that RNS-positivity (hazard ratio [HR] 6.007, P = 0.021) and histotype B2/B3 thymoma (HR 4.611, P = 0.048) were associated with OMG conversion. Thymic hyperplasia and stage I thymoma (HR 0.300, P = 0.026) were associated with OMG CSR after thymectomy.
CONCLUSION
For OMG patients after thymectomy, RNS-positivity and histotype B2/B3 thymoma are independent predictors of conversion to GMG. On the other hand, thymic hyperplasia and stage I thymoma independently predict CSR.
背景
几项回顾性研究已经确定了与非手术患者眼肌型重症肌无力（OMG）进展相关的风险因素。然而，胸腺瘤切除术后 OMG 的结果尚未得到充分研究。本研究旨在探讨胸腺瘤切除术后 OMG 预后的临床预测因素。
方法
我们对 2012 年 1 月至 2021 年 12 月在我院接受胸腺瘤切除术的 OMG 患者进行了回顾性分析。采用 Kaplan-Meier 和 Cox 比例风险回归分析评估临床特征与预后之间的关系。主要结局指标为 OMG 转化、完全稳定缓解（CSR）和临床改善。
结果
确定了 58 例用于转换分析的患者。13 例（22.4%）在症状出现后 12.7（3-37.3）个月时发展为全身性重症肌无力（GMG）。重复神经刺激（RNS）阳性与 GMG 转化风险增加相关（P=0.002）。B2/B3 胸腺瘤患者比增生和 AB/B1 胸腺瘤患者发生转化的风险更高（P=0.002）。52 例患者符合 CSR 和改善标准。16 例（30.8%）在胸腺瘤切除术后 28.7（15-54）个月达到 CSR。15 例（28.8%）在最后一次随访时显示临床改善。达到 CSR 的患者发病年龄较小（P=0.022），乙酰胆碱受体抗体阳性率较低（P=0.029）。组织学上，胸腺增生和 I 期胸腺瘤患者比 II/III 期胸腺瘤患者更有可能达到 CSR（P=0.010）。多变量分析显示，RNS 阳性（危险比[HR]6.007，P=0.021）和 B2/B3 胸腺瘤（HR 4.611，P=0.048）与 OMG 转化相关。胸腺增生和 I 期胸腺瘤（HR 0.300，P=0.026）与胸腺瘤切除术后 OMG 的 CSR 相关。
结论
对于胸腺瘤切除术后的 OMG 患者，RNS 阳性和 B2/B3 胸腺瘤是 GMG 转化的独立预测因素。另一方面，胸腺增生和 I 期胸腺瘤独立预测 CSR。
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