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

被引：0
|
作者：
Alexandre Belot
Rolando Cimaz
机构：
[1] Hôpital Femme Mère Enfant,Pediatric nephrology and rheumatology Unit
[2] Lyon,Rheumatology Unit, Anna Meyer Children's Hospital
[3] Bron,undefined
[4] Université de Lyon,undefined
[5] University of Florence,undefined
来源：
Pediatric Rheumatology | / 10卷
关键词：
SLE genetics; Mendelian; Complement deficiency; Interferon-alpha; Pediatrics;
D O I：
暂无
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学科分类号：
摘要：
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.
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