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- Title
The role of complement in the development of systemic lupus erythematosus.
- Authors
Manderson, Anthony P; Botto, Marina; Walport, Mark J
- 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 inflamed 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.
- Publication
Annual review of immunology, 2004, Vol 22, p431
- ISSN
0732-0582
- Publication type
Journal Article
- DOI
10.1146/annurev.immunol.22.012703.104549