Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster, Germany.
Department of Dermatology, Venerology, Allergology, and Phlebology, Johannes-Wesling-Hospital Minden, University Hospital Ruhr University of Bochum, Minden, Germany.
Front Immunol. 2020 Aug 28;11:2099. doi: 10.3389/fimmu.2020.02099. eCollection 2020.
Autoimmune adverse events are the most relevant risks of alemtuzumab therapy. We present a patient with relapsing-remitting multiple sclerosis, who developed adult-onset Still's disease (AOSD) following alemtuzumab treatment.
The patient suffered from sore throat, swallowing difficulties, high spiking quotidian fever, generalized skin rash, arthritis, and myalgia 2 months after the second course of alemtuzumab. Laboratory tests revealed elevated acute-phase reactants, anemia, neutrophilic leukocytosis, and thrombocytosis. Serum calprotectin, interleukin-2, and interleukin-6 levels were strongly increased. Autoimmune, rheumatic, neoplastic, infectious, and granulomatous disorders were excluded. The NLRP1 and NLRP3 gene test, which was performed under the presumption of a cryopyrin-associated autoinflammatory syndrome, was negative. Based on the Yamaguchi and Fautrel criteria, and supported by the histological findings from a skin biopsy of the rash, the diagnosis of AOSD was established. Therapy with the anti-IL-1 agent (anakinra) led to a significant improvement of symptoms and blood parameters. However, anakinra had to be converted to rituximab due to generalized drug eruption. Following therapy with rituximab, the patient has fully recovered.
The current case highlights AOSD as another rare and potentially life-threatening secondary autoinflammatory/autoimmune event following alemtuzumab treatment.
自身免疫不良反应是阿仑单抗治疗最相关的风险。我们报告了一例接受阿仑单抗治疗后发生成人Still 病（AOSD）的复发性缓解型多发性硬化症患者。
该患者在接受第二疗程阿仑单抗治疗 2 个月后出现咽痛、吞咽困难、高热、全身皮疹、关节炎和肌痛。实验室检查显示急性期反应物升高、贫血、中性粒细胞增多和血小板增多。血清钙卫蛋白、白细胞介素 2 和白细胞介素 6 水平明显升高。排除了自身免疫性、风湿性、肿瘤性、感染性和肉芽肿性疾病。鉴于存在 Cryopyrin 相关自身炎症综合征的假定，进行了 NLRP1 和 NLRP3 基因检测，但结果为阴性。根据 Yamaguchi 和 Fautrel 标准，并结合皮疹皮肤活检的组织学发现，诊断为 AOSD。采用抗白细胞介素 1 药物（阿那白滞素）治疗后，症状和血液参数显著改善。然而，由于全身性药物疹，不得不将阿那白滞素转换为利妥昔单抗。利妥昔单抗治疗后，患者完全康复。
本病例强调了 AOSD 是阿仑单抗治疗后另一种罕见且潜在危及生命的继发性自身炎症/自身免疫事件。
