Frémeaux-Bacchi Véronique, Dragon-Durey Marie-Agnès, Blouin Jacques, Mouthon Luc, Fridman Wolf Herman
Service d'Immunologie Biologique, Hôpital Européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, 20-40, rue Leblanc, 75908 Paris Cedex 15.
Ann Med Interne (Paris). 2003 Dec;154(8):529-40.
The complement system plays an important role in defence of the host against infection and in the clearance of immune complexes. Defects in complement proteins are often associated with infections or auto/immune complex diseases. Investigation of complement is useful for diagnosis and following of auto-immune diseases. The aim of this Article is to provide an overview of important applications of complement in medicine, emphasizing the role of complement in pathogenesis and the usefulness of measurements of complement proteins in diagnosis and assessment of the evolution of disease states. Emphasis has been placed on practical applications and understanding basic mechanisms of disease. The best screen for complement deficiencies or significant activation is the CH50, which measures total classical pathway activity and the measurement of C3 and C4. The absence or decrease of multiple components is usually due to consumption of complement. Complete lack of CH50 associated with normal C3 antigen is a strong indication for complement deficiency and should be followed up with further tests to determine which component is missing.
补体系统在宿主抗感染防御及免疫复合物清除过程中发挥着重要作用。补体蛋白缺陷常与感染或自身免疫/免疫复合物疾病相关。补体研究对于自身免疫性疾病的诊断及病情监测具有重要意义。本文旨在综述补体在医学中的重要应用，着重阐述补体在发病机制中的作用以及补体蛋白检测在疾病诊断和病情演变评估中的实用性。重点在于实际应用及对疾病基本机制的理解。补体缺陷或显著激活的最佳筛查指标是CH50，它可检测总经典途径活性以及C3和C4的水平。多种成分的缺失或减少通常是由于补体的消耗。CH50完全缺乏而C3抗原正常强烈提示补体缺陷，应进一步进行检测以确定缺失的是哪种成分。
