Systemic lupus erythematosus and complement deficiency: clues to a novel role for the classical complement pathway in the maintenance of immune tolerance

• Published in: Immunopharmacology Vol. 42; no. 1-3; pp. 47 - 52
• Main Authors: Navratil, Jeannine S, Korb, Laura C, Ahearn, Joseph M
• Format: Book Review Conference Proceeding Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 01-05-1999; Elsevier
• Subjects: Animals; Biological and medical sciences; C1q deficiency; Complement C1q - deficiency; Complement Pathway, Classical - immunology; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Humans; Immune Tolerance; Immunobiology; Immunological tolerance; Lupus Erythematosus, Systemic - blood; Lupus Erythematosus, Systemic - immunology; Systemic lupus erythematosus; Immune tolerance; Systemic lupus erythematosus; C1q deficiency; Human; Immunopathology; Connective tissue disease; Skin disease; Immune response; Rodentia; Autoimmune disease; Review; Complement C1q; Vertebrata; Mammalia; Autoantigen; Mouse; Cell death; Systemic disease; Lupus erythematosus; Disseminated; Apoptosis
• ISSN: 0162-3109
• DOI: 10.1016/S0162-3109(99)00018-1

Complete deficiency of C1q, the first component of the classical pathway of complement activation, is almost invariably associated with the development of systemic lupus erythematosus. Understanding why complement deficiency results in the specific autoimmune phenotype of SLE may provide valuable clues to the role of complement in the maintenance of immune tolerance. The following review will focus on the characteristics of complement-deficient SLE and the experimental evidence in support of our hypothesis that C1q may critically influence the immune response to self-antigen contained within surface blebs generated by apoptotic cells.