Cadranel J, Bouvry D, Wislez M
Service de Pneumologie et de Réanimation Respiratoire, Hôpital Tenon, AP-HP, Paris, France.
Rev Mal Respir. 2003 Feb;20(1 Pt 1):126-33.
Sporadic common variable immunodeficiency (CVID) is the most frequent cause of primary humoral immunodeficiency observed in adults. Respiratory complications occur frequently.
Recurrent bacterial pneumonia and bronchial suppuration are the most frequent complications whilst reactive interstitial pneumonitis (lymphoid interstitial pneumonia and sarcoid-like granulomatosis) and pulmonary lymphoma involvement are rarer. Treatment is based on replacement infusions of gamma globulin (Ig-IV). Although treatment has been shown to reduce the frequency of pulmonary infections, respiratory complications are still responsible for one third of late mortality in CVID.
Type and frequency of respiratory survey has to be defined, as well as the indication for other treatments than Ig-IV.
Protein electrophoresis must be done in a young adult with recurrent low respiratory tract infections and/or diffuse bronchectasis. Hypogammaglobulinemia should result in immunological and hematological investigations which may help to the diagnosis of CVID and to the beginning of the adequate treatment.
散发性普通可变免疫缺陷(CVID)是成人中观察到的原发性体液免疫缺陷的最常见原因。呼吸并发症频繁发生。
复发性细菌性肺炎和支气管化脓是最常见的并发症,而反应性间质性肺炎(淋巴间质性肺炎和类肉瘤样肉芽肿病)和肺淋巴瘤累及则较为罕见。治疗基于γ球蛋白(静脉注射免疫球蛋白)替代输注。尽管治疗已被证明可降低肺部感染的频率,但呼吸并发症仍是CVID晚期死亡率的三分之一的原因。
必须确定呼吸检查的类型和频率,以及除静脉注射免疫球蛋白之外的其他治疗的适应症。
对于反复发生下呼吸道感染和/或弥漫性支气管扩张的年轻成人,必须进行蛋白电泳。低丙种球蛋白血症应进行免疫学和血液学检查,这可能有助于CVID的诊断和开始适当的治疗。