Wood P, Stanworth S, Burton J, Jones A, Peckham D G, Green T, Hyde C, Chapel H
Department of Clinical Immunology, St James's University Hospital, Leeds, UK.
Clin Exp Immunol. 2007 Sep;149(3):410-23. doi: 10.1111/j.1365-2249.2007.03432.x. Epub 2007 Jun 12.
The primary purpose of this systematic review was to produce an evidence-based review of the literature as a means of informing current clinical practice in the recognition, diagnosis and management of patients with suspected primary antibody deficiency. Randomized controlled trials (RCTs) were identified from a search of MEDLINE, EMBASE, The Cochrane Library, DARE (CRD website) and CINAHL by combining the search strategies with The Cochrane Collaboration's validated RCT filter. In addition, other types of studies were identified in a separate search of MEDLINE and EMBASE. Patients at any age with recurrent infections, especially in the upper and lower respiratory tracts, should be investigated for possible antibody deficiency. Replacement therapy with immunoglobulin in primary antibody deficiencies increases life expectancy and reduces infection frequency and severity. Higher doses of immunoglobulin are associated with reduced infection frequency. Late diagnosis and delayed institution of immunoglobulin replacement therapy results in increased morbidity and mortality. A wide variety of organ-specific complications can occur in primary antibody deficiency syndromes, including respiratory, gastroenterological, hepatic, haematological, neurological, rheumatological and cutaneous. There is an increased risk of malignancy. Some of these complications appear to be related to diagnostic delay and inadequate therapy. High-quality controlled trial data on the therapy of these complications is generally lacking. The present study has identified a number of key areas for further research, but RCT data, while desirable, is not always obtained easily for rare conditions. Few data from registries or large case-series have been published in the past 5 years and a greater focus on international collaboration and pooling of data is needed.
本系统评价的主要目的是对文献进行循证综述，以为目前疑似原发性抗体缺陷患者的识别、诊断和管理方面的临床实践提供参考。通过将检索策略与Cochrane协作网验证的随机对照试验（RCT）过滤器相结合，从MEDLINE、EMBASE、Cochrane图书馆、DARE（CRD网站）和CINAHL中检索出RCT。此外，在对MEDLINE和EMBASE的单独检索中识别出其他类型的研究。任何年龄有反复感染，尤其是上、下呼吸道感染的患者，都应调查是否可能存在抗体缺陷。原发性抗体缺陷患者使用免疫球蛋白替代疗法可延长预期寿命，降低感染频率和严重程度。较高剂量的免疫球蛋白与较低的感染频率相关。诊断延迟和免疫球蛋白替代疗法启动延迟会导致发病率和死亡率增加。原发性抗体缺陷综合征可出现多种器官特异性并发症，包括呼吸、胃肠、肝脏、血液、神经、风湿和皮肤方面的并发症。恶性肿瘤风险增加。其中一些并发症似乎与诊断延迟和治疗不足有关。关于这些并发症治疗的高质量对照试验数据普遍缺乏。本研究确定了一些需要进一步研究的关键领域，但对于罕见疾病，虽然理想，但并非总能轻易获得RCT数据。在过去5年中，来自登记处或大型病例系列的数据很少发表，需要更加关注国际合作和数据汇总。
