Infections in 252 patients with common variable immunodeficiency
Infections in 252 patients with common variable immunodeficiency
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DOI：
10.1086/587669
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发表时间：
2008-05-15
影响因子：
11.8
通讯作者：
Debre, Patrice
中科院分区：
文献类型：
--
作者：
Oksenhendler, Eric;Gerard, Laurence;Debre, Patrice
背景资料。常见变异型免疫缺陷的特征是反复感染和免疫球蛋白产生缺陷。DEFI法国国家研究前瞻性地纳入了患有原发性低丙种球蛋白血症的成年患者。对纳入前的临床事件进行回顾性分析。从2004年4月到2007年4月，纳入了341名患者，其中252人被诊断为常见变量免疫缺陷；其中110人为男性，142人为女性，228人为白人。首次出现症状的中位年龄为19岁。诊断为共同变量免疫缺陷的中位年龄为33.9岁。1990年之前有首发症状的138名患者的中位诊断延迟时间为15.6年，从1990年到研究时间的114名首发症状患者的中位诊断延迟时间为2.9年。最常见的最初症状是上呼吸道感染：支气管炎(38%的患者)、鼻窦炎(36%)、肺炎(31%)和/或支气管扩张(14%)。总体而言，240名患者有呼吸道症状。肺炎147例，肺炎链球菌46例，流感嗜血杆菌17例。118例患者报告有反复或慢性腹泻。在血清免疫球蛋白A检测不到的患者中，贾第虫(35例)、沙门氏菌(19例)和弯曲杆菌(19例)感染更常见(P<；.001)。16名患者出现机会性感染。持续感染和使用抗生素与记忆转换B细胞严重缺陷相关(P=0.003)，而与免疫球蛋白G谷值无关(P=0.55)。尽管在过去的十年中有所减少，但常见变量免疫缺陷的延迟诊断仍然是不可接受的。上呼吸道感染或肺炎的复发应对血清免疫球蛋白进行系统评估。
Background. Common variable immunodeficiency is characterized by recurrent infections and defective immunoglobulin production.Methods. The DEFI French national study prospectively enrolled adult patients with primary hypogammaglobulinemia. Clinical events before inclusion were retrospectively analyzed at that time.Results. From April 2004 through April 2007, 341 patients were enrolled, 252 of whom had received a diagnosis of common variable immunodeficiency; of those, 110 were male, 142 were female, and 228 were white. The median age at first symptoms was 19 years. The median age at common variable immunodeficiency diagnosis was 33.9 years. The median delay for diagnosis was 15.6 years for the 138 patients with initial symptoms before 1990 and 2.9 years for the 114 patients with initial symptoms from 1990 to the time of the study. The most frequent initial symptoms were upper respiratory tract infections: bronchitis (in 38% of patients), sinusitis (36%), pneumonia (31%), and/or bronchiectasis (14%). Overall, 240 patients had respiratory symptoms. Pneumonia was reported in 147 patients; Streptococcus pneumoniae and Haemophilus influenzae were documented in 46 and 17 cases, respectively. Recurrent or chronic diarrhea was reported in 118 patients. Giardia (35 cases), Salmonella (19), and Campylobacter (19) infections were more frequent in patients with undetectable serum immunoglobulin A (P < .001). Sixteen patients developed opportunistic infections. Persistent infections and requirement for antibiotics despite immunoglobulin substitution correlated with severe defect of memory switched B cells (P = .003) but not with immunoglobulin G trough levels (P = .55).Conclusion. Although reduced within the past decade, the delay of diagnosis of common variable immuno-deficiency remains unacceptable. Recurrence of upper respiratory tract infection or pneumonia should lead to systematic evaluation of serum immunoglobulin.