Section of Paediatrics, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Department of Clinical Immunology and Allergology, I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
Front Immunol. 2019 Jul 17;10:1700. doi: 10.3389/fimmu.2019.01700. eCollection 2019.
Chronic spontaneous urtcaria (CSU) can represent the leading sign of a wide spectrum of systemic diseases, including primary immunodeficiencies. We describe the case of a young adult female with coexisting CSU and common variable immunodeficiency (CVID) successfully treated with omalizumab. The patient, with a history of recurrent respiratory infections during childhood, was referred to clinical attention due to the development of refractory CSU. During the diagnostic workup for the research of secondary causes of urticaria, an immunological assessment was performed, showing markedly reduced levels of IgG and IgM, poor antibody response against vaccinating antigens in absence of a T cellular deficiency. Therefore, the diagnosis of CVID was posed. Despite the immunoglobulin replacement and a trial with intravenous immunoglobulin at immunomodulatory dosage, the patient continued to experience severe urticaria, with significant impairment in the quality of life. After 2 years from the diagnosis of CVID, a treatment with omalizumab was started, showing complete remission of cutaneous symptoms after the first injection. The drug was well-tolerated, and the patient did not experience adverse effects during a 12-months follow-up.
慢性自发性荨麻疹 (CSU) 可作为多种系统性疾病的主要表现，包括原发性免疫缺陷病。我们描述了一例年轻女性患者，其同时患有 CSU 和普通变异性免疫缺陷病 (CVID)，经奥马珠单抗治疗后获得良好疗效。该患者在儿童时期反复发生呼吸道感染，因难治性 CSU 就诊。在对荨麻疹的继发性病因进行研究的诊断过程中，进行了免疫学评估，结果显示 IgG 和 IgM 水平显著降低，接种抗原后抗体反应不良，且不存在 T 细胞缺陷。因此，诊断为 CVID。尽管进行了免疫球蛋白替代治疗和免疫调节剂量的静脉注射免疫球蛋白治疗，但患者仍持续出现严重荨麻疹，生活质量显著受损。在诊断为 CVID 后 2 年，开始使用奥马珠单抗治疗，首次注射后皮肤症状完全缓解。该药物耐受性良好，在 12 个月的随访期间未发生不良反应。
