Lin Joann H, Liebhaber Myron, Roberts Robert L, Dyer Zeb, Stiehm E Richard
Department of Pediatrics, Division of Immunology, Allergy, and Rheumatology, and David Geffen School of Medicine at UCLA, Los Angeles, California 90095, USA.
J Allergy Clin Immunol. 2006 Apr;117(4):878-82. doi: 10.1016/j.jaci.2006.01.034.
Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia, poor antibody responses, and recurrent bacterial infections, usually of the sinorespiratory tract. A not uncommon complication is granuloma of the lungs, spleen, liver, and/or skin. We report the case of an 18-year-old boy with CVID and chronic granulomas of the left arm (since 13 years of age) refractory to treatment with antibiotics, intravenous immunoglobulin, antifungal agents, systemic and intralesional steroids, IFN-gamma, cyclosporine, methotrexate, hydroxychloroquine, localized radiation therapy, and surgical excision. The lesions improved after treatment with the systemic administration of the TNF-alpha inhibitor etanercept for 1 year. Etanercept prevents soluble TNF from binding to its cell membrane receptor, leading to inhibition of its inflammatory cascade. We recommend further trials of etanercept in patients with CVID with noninfectious recalcitrant granulomas.
普通可变免疫缺陷（CVID）是一种原发性免疫缺陷，其特征为低丙种球蛋白血症、抗体反应不佳以及反复发生细菌感染，通常累及上呼吸道。肺部、脾脏、肝脏和/或皮肤出现肉芽肿是一种并不罕见的并发症。我们报告了一例18岁男孩，患有CVID且左臂慢性肉芽肿（自13岁起），对抗生素、静脉注射免疫球蛋白、抗真菌药物、全身及病灶内类固醇、干扰素-γ、环孢素、甲氨蝶呤、羟氯喹、局部放射治疗及手术切除均无反应。使用肿瘤坏死因子-α抑制剂依那西普全身给药治疗1年后，病变有所改善。依那西普可阻止可溶性肿瘤坏死因子与其细胞膜受体结合，从而抑制其炎症级联反应。我们建议对患有CVID且伴有难治性非感染性肉芽肿的患者进一步进行依那西普试验。
