Common variable immunodeficiency diagnosed during the treatment of bronchial asthma: Unusual cause of wheezing.

Common variable immunodeficiency diagnosed during the treatment of bronchial asthma: Unusual cause of wheezing.
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DOI:
10.1016/j.rmcr.2015.07.002
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发表时间:
2015
影响因子:
1.1
通讯作者:
Tamaoki J
Tamaoki J
中科院分区:
其他
文献类型:
--
作者:
Akaba T;Kondo M;Toriyama M;Kubo A;Hara K;Yamada T;Yoshinaga K;Tamaoki J

文献摘要

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常见变异性免疫缺陷(CVID)是成人和儿童中最常见的原发性免疫缺陷。我们在此报告一个CVID的病例,由于喘息发作和相对较晚的发作而被误诊为哮喘。患者女,51岁,反复上、下呼吸道感染2年。她反复喘息发作,被视为感染引发的哮喘加重。她被转诊到我院接受检查和治疗。肺功能检查显示β-肾上腺素能受体激动剂对FEV 1无逆转作用,但V50/V25增加。胸部CT示轻至中度支气管壁增厚和支气管扩张。之后,她患上了肺炎,每月两次喘息发作,并强烈怀疑免疫缺陷。她的血液检查显示所有类别的免疫球蛋白明显减少,几乎缺乏记忆B细胞、NKT细胞和浆细胞样树突状细胞。她被诊断为CVID,并接受了丙种球蛋白替代治疗。此后,她的喘息发作与感染显着改善。由于CVID诊断的延迟可能导致低死亡率和发病率,因此在因反复气道感染而频繁出现哮喘样症状的患者中应考虑CVID的可能性。
Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency in adults and children. We herein report a case of CVID, who was misdiagnosed with asthma due to wheezing episodes and relatively late onset. A 51-year-old woman had suffered from recurrent upper and lower airway infection for recent 2 years. She repeated wheezing attacks and was treated as asthma exacerbation triggered by infection. She was referred to our hospital for investigation and treatment. Lung function tests showed no reversibility of FEV1 by β-adrenergic agonist, but the increase of V50/V25. Chest CT showed slight to moderate bronchial wall thickening and bronchiectasis. After that, she suffered from pneumonia with wheezing attacks twice a month, and immunodeficiency was strongly suspected. Her blood tests showed marked decreases of all classes of immunoglobulin and nearly lack of memory B cells, NKT cells and plasmacytoid dendritic cells. She was diagnosed with CVID, and was treated with replacement of gammaglobulin. Thereafter, her wheezing episodes with infection were remarkably improved. Because the delay of diagnosis with CVID likely causes poor mortality and morbidity, a possibility of CVID should be considered in patients with frequent asthma-like symptoms due to recurrent airway infection.