Department of Neurology, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, NG7 2UH, UK.
Academic Neurology Group, Division of Clinical Neuroscience, University of Nottingham, Nottingham, UK.
J Neurol. 2024 Sep;271(9):6220-6226. doi: 10.1007/s00415-024-12592-x. Epub 2024 Jul 30.
There are only a few studies exploring post-thymectomy outcome in patients with acetylcholine receptor antibody (AChR-Ab)-positive generalised myasthenia gravis (MG).
To assess the predictors of outcome in patients with AChR-Ab-positive generalised MG who underwent thymectomy.
A retrospective study of 53 patients from a single neuroscience centre in the UK.
The mean disease duration from diagnosis was 6.2 ± 4.3 years. Pre-thymectomy, 37 patients had mild weakness affecting muscles other than ocular muscles, 11 patients had moderate weakness and 5 patients had severe weakness. 27/53 patients had thymoma. Post-thymectomy (mean duration of 5.7 ± 4.2 years), 34 patients (64%) had a good outcome characterised by Myasthenia Gravis Foundation of America Post-Intervention Status of complete stable remission (no symptoms or signs of MG for at least 1 year without any therapy) or pharmacological remission (no symptoms or signs of MG with some form of therapy) or minimal manifestations (no symptoms of functional limitations from MG but weakness on examination of some muscles with or without some form of therapy) on last follow-up visit. Having thymomatous or non-thymomatous MG did not predict the outcome. The only variable that did predict outcome was pre-thymectomy disease severity; patients with mild weakness before thymectomy had a favourable outcome. We found an accuracy of 83% predicting outcome (95% confidence interval (CI) 60%, 100%) with a sensitivity of 84% (95% CI 68%, 94%) and specificity of 81% (95% CI 54%, 96%).
Disease severity before thymectomy predicts outcome in patients with AChR-Ab-positive generalised MG.
仅有少数研究探讨了乙酰胆碱受体抗体（AChR-Ab）阳性全身型重症肌无力（MG）患者胸腺切除术后的预后。
评估 AChR-Ab 阳性全身型 MG 患者行胸腺切除术的预后预测因素。
对英国单中心神经科学中心的 53 例患者进行回顾性研究。
从诊断到发病的平均病程为 6.2±4.3 年。术前，37 例患者有眼外肌以外肌肉的轻度无力，11 例患者有中度无力，5 例患者有重度无力。53 例患者中有 27 例为胸腺瘤。术后（平均病程 5.7±4.2 年），34 例（64%）患者预后良好，表现为美国重症肌无力基金会干预后状态：完全稳定缓解（至少 1 年无任何治疗，无 MG 症状或体征）或药理学缓解（无 MG 症状或体征，用某种形式的治疗）或最小表现（无 MG 功能受限的症状，但在某些肌肉检查时存在无力，用或不用某种形式的治疗）。有胸腺瘤或无胸腺瘤的 MG 并不能预测预后。唯一能预测预后的变量是术前疾病严重程度；术前有轻度无力的患者预后良好。我们发现预测预后的准确率为 83%（95%置信区间（CI）60%，100%），灵敏度为 84%（95% CI 68%，94%），特异性为 81%（95% CI 54%，96%）。
术前疾病严重程度可预测 AChR-Ab 阳性全身型 MG 患者的预后。
