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. 2012 Aug 10;10(1):21.
doi: 10.1186/1546-0096-10-21.

Monogenic forms of systemic lupus erythematosus: new insights into SLE pathogenesis

Alexandre Belot  1 , Rolando Cimaz
Affiliations

Monogenic forms of systemic lupus erythematosus: new insights into SLE pathogenesis

Alexandre Belot et al. Pediatr Rheumatol Online J. .

Abstract

The pathogenesis of Systemic Lupus Erythematosus (SLE) is complex and remains poorly understood. Infectious triggers, genetic background, immunological abnormalities and environmental factors are all supposed to interact for the disease development. Familial SLE as well as early-onset juvenile SLE studies make it possible to identify monogenic causes of SLE. Identification of these rare inherited conditions is of great interest to understand both SLE pathogenesis and molecular human tolerance mechanisms. Complement deficiencies, genetic overproduction of interferon-α and apoptosis defects are the main situations that can lead to monogenic SLE.Here, we review the different genes involved in monogenic SLE and highlight their importance in SLE pathogenesis.

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Figures

Figure 1
Figure 1
Schematic views of monogenic SLE pathogenesis.
See this image and copyright information in PMC

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