Rheumatology 2000; 39: 133-141
© 2000 British Society for Rheumatology
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Links between complement abnormalities and systemic lupus erythematosus
Systemic Lupus Erythematosus/Series Editors: D. Isenberg and C. Gordon
Rheumatology Section, Department of Medicine, Imperial College School of Medicine, Hammersmith Campus, Du Cane Road, London W12 0NN, UK
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Introduction |
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Complement is an important effector pathway of innate immunity. In this review the role of complement in the pathogenesis of systemic lupus erythematosus (SLE) is described. There are important associations between both inherited and acquired complement component deficiencies and SLE. These have focused attention on identifying the relevant physiological role of the classical pathway of complement activation that appears to protect against the development of SLE. There is also unequivocal evidence that major histocompatibility complex (MHC) genes play an important role in determining both disease susceptibility and phenotype in SLE and its subsets. Accordingly, the role of complement genes located in the class III region (C4A, C4B and C2) in the genetic susceptibility to SLE is reviewed. The utility of the measurement of complement levels in monitoring disease activity and the clinical significance of hypocomplementaemia are discussed. Homozygous C1q deficiency is the strongest genetic susceptibility factor for SLE that has
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Homozygous classical pathway component deficiency and SLE |
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C4 and C2 null alleles and SLE |
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C4 null alleles
C2 null alleles
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Acquired deficiencies of complement proteins and SLE |
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C1 inhibitor deficiency
C3 nephritic factor
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Complement and disease activity |
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Infection and hypocomplementaemia in SLE
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The role of complement in the pathogenesis of SLE |
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Role of complement in immune complex processing and immune complex-mediated tissue inflammation
C1q- and receptor-mediated clearance of apoptotic cells
Complement and B cell tolerance in SLE
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Conclusion |
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Acknowledgments |
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Notes |
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References |
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