Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis
Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis
复制标题
DOI：
10.1111/bjd.13525
复制
发表时间：
2015-04-01
影响因子：
10.3
通讯作者：
Zhu, X.
中科院分区：
文献类型：
--
作者：
Wang, R.;Li, J.;Zhu, X.
背景 副肿瘤性天疱疮 (PNP) 累及多个器官，但对其神经系统受累知之甚少。 目的 探讨重症肌无力 (MG) 患者的症状、预后和相关自身抗体谱，及其与 PNP 患者的相关性。 方法 对 58 例 PNP 患者进行肌无力症状和实验室证据评估。通过酶联免疫吸附测定检测血清中抗乙酰胆碱受体（AChR）、乙酰胆碱酯酶（AChE）、肌联蛋白、兰尼碱受体（RyR）和肌肉特异性激酶（MuSK）的自身抗体。寻常型天疱疮 (PV)、落叶型天疱疮 (PF)、结缔组织病 (CTD) 和非 PNP MG (NP-MG) 患者以及健康供者作为对照。还对 PNP 中的这些自身抗体是否存在呼吸困难或肌无力进行了比较。 Cox 回归和对数秩检验用于生存分析。结果 总体而言，39% 的 PNP 患者出现肌无力，35% 被诊断为 MG。此外，与 NP-MG 类似（分别为 33% 和 17%，P > 0.05），35% 的人抗 AChR 呈阳性，28% 的人有抗 AChE 抗体。然而，所有 PV、PF 和 CTD 患者以及健康供体的结果均为阴性（P < 0.005）。其他抗体在组间没有表现出显着差异。伴有呼吸困难的PNP患者抗AChR和抗AChE抗体水平显着升高，而伴有肌无力的PNP患者抗AChR、抗肌联蛋白和抗RyR水平显着升高（P < 0.05）。然而，这些自身抗体的水平和阳性率在伴有 Castleman 病和胸腺瘤的 PNP 之间没有显着差异。尽管抗 AChE 水平影响生存时间（P = 0.027，比值比 3.14），但 MG 并发症并不影响 PNP 的总生存率。结论 MG 是 PNP 的并发症。抗 AChR 和抗 AChE 抗体在 PNP 患者中很明显，尤其是呼吸困难的患者。
Background Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement.Objectives To investigate the symptoms, prognosis and profiles of associated autoantibodies in myasthenia gravis (MG), and their correlations in patients with PNP.Methods Fifty-eight patients with PNP were assessed for myasthenic symptoms and laboratory evidence. Serum autoantibodies against acetylcholine receptor (AChR), acetylcholinesterase (AChE), titin, ryanodine receptor (RyR) and muscle-specific kinase (MuSK) were measured by enzyme-linked immunosorbent assay. Patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue disease (CTD) and non-PNP MG (NP-MG), and healthy donors, served as controls. These autoantibodies in PNP were also compared in the presence or absence of dyspnoea or muscle weakness. Cox regression and log-rank tests were used for survival analysis.Results Overall 39% of patients with PNP experienced muscle weakness, and 35% were diagnosed with MG. Moreover, 35% had positive anti-AChR and 28% had anti-AChE antibodies, similarly to NP-MG (33% and 17%, respectively, P > 0.05). However, both were negative in all patients with PV, PF and CTD and healthy donors (P < 0.005). No other antibodies showed significant differences among groups. Anti-AChR and anti-AChE antibody levels were significantly increased in patients with PNP with dyspnoea, while anti-AChR, anti-titin and anti-RyR were significantly increased in patients with PNP with muscle weakness (P < 0.05). Nevertheless, levels and positive rates of these autoantibodies showed no significant differences between PNP with Castleman disease and thymoma. Although anti-AChE levels impacted survival duration (P = 0.027, odds ratio 3.14), MG complications did not affect the overall survival percentage in PNP.Conclusions MG is a complication of PNP. Anti-AChR and anti-AChE antibodies are prominent in patients with PNP, especially those with dyspnoea.