Objective

To report the efficacy of rituximab plus belimumab in patients with refractory cryoglobulinemia vasculitis (CV).

Methods

Belimumab was administered intravenously at a dose of 10 mg/kg on days 0, 14, 28 and then every month in association with rituximab in 4 patients with refractory CV. Demographic, clinical and laboratory characteristics, treatment modalities and outcomes were recorded.

Results

All patients had type II IgM Kappa cryoglobulinemia, which was associated with primary Sjögren syndrome (n = 1), hepatitis C virus infection (n = 1), and essential (n = 2). Main manifestations of CV included purpura (n = 4), arthralgia and peripheral neuropathy (n = 3), and glomerulonephritis and skin ulcers (n = 1). In all cases, CV was refractory and/or relapse following rituximab. Intravenous belimumab infusion along with rituximab resulted in rapid clinical response in the four patients. Osteitis and recurrent urinary tract infections occurred in two patients.

Conclusion

Belimumab along with rituximab showed promising results in refractory patients with CV.

中文翻译：

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利妥昔单抗加贝利木单抗治疗非感染性难治性冷球蛋白血症血管炎：一项初步研究

客观的

报告利妥昔单抗联合贝利木单抗治疗难治性冷球蛋白血症血管炎（CV）患者的疗效。

方法

在 4 名难治性 CV 患者中，贝利木单抗在第 0、14、28 天以 10 mg/kg 的剂量静脉内给药，然后每月与利妥昔单抗联合给药。记录人口统计学、临床和实验室特征、治疗方式和结果。

结果

所有患者均患有 II 型 IgM Kappa 冷球蛋白血症，这与原发性干燥综合征 (n = 1)、丙型肝炎病毒感染 (n = 1) 和必需 (n = 2) 相关。CV 的主要表现包括紫癜（n = 4）、关节痛和周围神经病变（n = 3）以及肾小球肾炎和皮肤溃疡（n = 1）。在所有情况下，利妥昔单抗后 CV 均难治和/或复发。静脉注射贝利木单抗和利妥昔单抗导致 4 名患者的临床反应迅速。两名患者发生骨炎和复发性尿路感染。

结论

贝利木单抗和利妥昔单抗在难治性 CV 患者中显示出有希望的结果。