Abstract

This chapter reveals the relationship between deficiency of the C1 or C2 chromosomes with systemic lupus erythematosus. It begins with some background data on the structure and function of the class III complement genes and their protein products. Following this, it reviews the older data relative to the complement's long-appreciated role in immune complex (IC) handling. The new tools in this field, namely knockout mice, have been helpful in establishing an important role for the complement in innate and adaptive immunity as well as in preventing autoimmunity. A problem faced by a C-deficient host revolves around the clearance of foreign antigens and damaged self. The lack of C3b deposition on an IC leads to impaired antigen localization such as to follicular areas of the spleen. This appears to be a critical factor in the poor antibody response of such individuals. In a C-deficient host, there are several avenues to a vasculitic state. Prolonged antigenemia results in conditions such as in hepatitis B and C infections. In a C-deficient host, even normal amounts of IC, in association with infections or associated with physiologic tissue turnover and cell death, have a greater likelihood of being inappropriately processed. The prevention of IC precipitation in vascular structures, their adherence to erythrocytes or leukocytes, and their transport to and deposition in the liver and spleen are all likely to be impaired. Finally, the chapter discusses some recent results on the complement's key role in instructing adaptive immunity and in processing cellular debris.

Original languageEnglish
Title of host publicationSystemic Lupus Erythematosus
PublisherElsevier Inc.
Pages21-45
Number of pages25
ISBN (Electronic)9780123749949
ISBN (Print)9780123749949
DOIs
StatePublished - 2011

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