Department of Neurology, Karolinska University Hospital, Stockholm, Sweden; Department of Clinical 7 Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Department of Neurology, Karolinska University Hospital, Stockholm, Sweden; Department of Clinical 7 Neuroscience, Karolinska Institutet, Stockholm, Sweden.
J Neuroimmunol. 2019 Nov 15;336:577028. doi: 10.1016/j.jneuroim.2019.577028. Epub 2019 Aug 23.
We present a 53-year-old woman who presented simultaneously with acute inflammatory demyelinating polyneuropathy, Graves' disease, leukocytoclastic vasculitis, elevated acetylcholine antibody receptor antibodies and a mediastinal mass. Thymectomy was performed and revealed a type A thymoma and the clinical picture and paraclinical findings were consistent with a thymoma-associated multi-autoimmune syndrome (TAMA). Beside prednisolone and plasmapheresis, the patient was treated with tocilizumab and rituximab. After surgical and immunomodulatory treatment with tocilizumab and rituximab the patient's condition slowly started to improve. TAMA is associated with a spectrum of autoantibodies and immune-mediated damage to multiple organs. Even if thymectomy is crucial for long term prognosis, aggressive immunomodulation should be considered early in the disease course, especially in cases showing involvement of the peripheral and/or central nervous system.
我们现介绍一位 53 岁女性,她同时患有急性炎症性脱髓鞘性多发性神经病、格雷夫斯病、白细胞碎裂性血管炎、乙酰胆碱受体抗体升高和纵隔肿块。进行了胸腺切除术,结果显示为 A 型胸腺瘤,且临床和辅助检查结果符合胸腺瘤相关的多自身免疫综合征(TAMA)。除泼尼松龙和血浆置换外,患者还接受了托珠单抗和利妥昔单抗治疗。在接受托珠单抗和利妥昔单抗的手术和免疫调节治疗后,患者的病情逐渐开始好转。TAMA 与一系列自身抗体和对多个器官的免疫介导损伤相关。即使胸腺切除术对长期预后至关重要,但在疾病早期就应考虑积极的免疫调节治疗,尤其是在出现外周和/或中枢神经系统受累的情况下。