aDivision of Rheumatology, Department of Internal Medicine bDivision of Allergy and Immunology, Departments of Internal Medicine and Pediatrics and Communicable Diseases, University of Michigan, Michigan, USA.
Curr Opin Rheumatol. 2017 May;29(3):228-233. doi: 10.1097/BOR.0000000000000377.
There are no established guidelines for evaluating and treating hypogammaglobulinemia in patients with rheumatic disease who receive B-cell depleting agents. The purpose of this article is to review findings in the work-up and treatment of common variable immunodeficiency (CVID) that can guide our evaluation of patients with autoimmune disease who develop hypogammaglobulinemia after rituximab/B-cell depleting therapy.
Infection rates are higher in rheumatic disease patients who develop hypogammaglobulinemia than those who do not. However, not all patients who develop hypogammaglobulinemia are at increased risk of developing infection after B-cell depleting therapy. Recent consensus statements have helped refine the diagnosis of impaired immune responses in patients with CVID, and can provide guidance for the diagnostic work-up and therapeutic decision making for patients with secondary drug induced hypogammaglobulinemia.
Based on findings in studies of CVID, assessment of vaccine response in patients with hypogammglogulinemia after rituximab therapy in the setting of recurrent infections can help predict propensity for infection and thus guide decision making with regards to intravenous immunoglobulin supplementation and retreatment with rituximab.
目前尚无针对接受 B 细胞耗竭剂治疗的风湿性疾病患者低丙种球蛋白血症评估和治疗的既定指南。本文旨在回顾常见可变免疫缺陷（CVID）患者在接受利妥昔单抗/ B 细胞耗竭治疗后出现低丙种球蛋白血症的研究结果，以指导我们对自身免疫性疾病患者进行评估。
与未发生低丙种球蛋白血症的风湿性疾病患者相比，发生低丙种球蛋白血症的患者感染率更高。然而，并非所有发生低丙种球蛋白血症的患者在接受 B 细胞耗竭治疗后都有更高的感染风险。最近的共识声明有助于完善 CVID 患者免疫反应受损的诊断，可为继发性药物诱导低丙种球蛋白血症患者的诊断和治疗决策提供指导。
基于 CVID 研究的结果，在反复感染的情况下，对利妥昔单抗治疗后低丙种球蛋白血症患者的疫苗反应进行评估有助于预测感染倾向，从而指导静脉免疫球蛋白补充和利妥昔单抗再治疗的决策。
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