Complement Deficiencies in Systemic Lupus Erythematosus

  • Angela R. Bryan
  • Eveline Y. WuEmail author
Autoimmunity (TK Tarrant, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Autoimmunity


The complement system is a major, multifunctional part of innate immunity and serves as a bridge between the innate and adaptive immune systems. It consists of more than 30 distinct proteins that interact with one another in a specific sequence. There are three pathways of complement activation: the classical, the lectin, and the alternative pathways. The three pathways are initiated by distinct mechanisms, but they all generate the same core set of effector molecules. Inherited complete deficiencies in complement components are generally very rare and predispose to infections and autoimmune disease. One of the better described associations is between deficiencies in early classical pathway components and the development of systemic lupus erythematosus. The goal of this review will be to discuss the associations between and the causal mechanisms of complement deficiencies and systemic lupus erythematosus.


Complement Complement deficiency Classical pathway Systemic lupus erythematosus Autoimmune disease 


Compliance with Ethics Guidelines

Conflict of Interest

Angela Bryan and Eveline Wu declare that they have no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Pediatric Rheumatology DivisionDuke University Children’s Health CenterDurhamUSA
  2. 2.Division of Pediatric Allergy/Immunology and RheumatologyUniversity of North Carolina at Chapel HillChapel HillUSA

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