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| The role of complement in the development of systemic lupus erythematosus |
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| Author(s): Manderson AP, Botto M, Walport MJ |
| Source: ANNUAL REVIEW OF IMMUNOLOGY Volume: 22 Pages: 431-456 Published: 2004 |
| Times Cited: 129 References: 153 |
| Abstract: Complement has both beneficial and deleterious roles in the pathogenesis of systemic lupus erythematosus (SLE). On the one hand, patients with SLE present with decreased complement levels and with complement deposition in inflammed tissues, suggestive of a harmful role of complement in the effector phase of disease. On the other hand, homozygous deficiency of any of the classical pathway proteins is strongly associated with the development of SLE. There are two main hypotheses to explain these observations. The first invokes an important role for complement in the physiological waste-disposal mechanisms of dying cells and immune complexes. The second hypothesis is based around the role of complement in determining the activation thresholds of B and T lymphocytes, with the proposal that complement deficiency causes incomplete maintenance of peripheral tolerance. These two hypotheses are not mutually exclusive. In addition, there is evidence for a contribution from other genetic factors in determining the phenotype of disease in the absence of complement. |
| Document Type: Review |
| Language: English |
| Reprint Address: Manderson, AP (reprint author), Univ London Imperial Coll Sci Technol & Med, Fac Med, Div Med, Rheumatol Sect, Hammersmith Campus, London W12 0NN, England |
Addresses:
1. Univ London Imperial Coll Sci Technol & Med, Fac Med, Div Med, Rheumatol Sect, London W12 0NN, England
2. The Wellcome Trust, London NW1 2BE, England |
| Publisher: ANNUAL REVIEWS, 4139 EL CAMINO WAY, PO BOX 10139, PALO ALTO, CA 94303-0139 USA |
| Subject Category: Immunology |
| IDS Number: 823HP |
| ISSN: 0732-0582 |
| DOI: 10.1146/annurev.immunol.22.012703.104549 |
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