Lupus and lupus-like syndromes View Full Text


Ontology type: schema:Chapter     


Chapter Info

DATE

1999

AUTHORS

Gwan Gyu Song , Martin Fleck , Jianguo Wu , Hui-Chen Hsu , Tong Zhou , John D. Mountz

ABSTRACT

The pathogenesis of systemic lupus erythematosus (SLE) is multifactorial and multi-genetic. Chronic inflammation associated with lupus is thought to be due to loss of self-tolerance due to molecular mimicry, environment trigger, hormonal factors or apoptosis defects. Defects in apoptosis will be the focus of this chapter. Defects in apoptosis can lead to abnormal clonal deletion of autoreactive cells or failure to downmodulate an inflammatory response. Although the Fas death domain family of molecules are the primary pathway for elimination of inflammatory cells, defects in these death domain molecules are rarely observed in lupus and lupus-like syndromes. Patients with autoimmune-lymphoproliferative (ALPS) syndrome disease have defects in Fas, and we have reported one patient with SLE that exhibits a mutation of Fas ligand. Other death domain family molecules such as death receptor 3 (DR3), DR4, DRS, Fas ligand 2 (FasL2) have not been studied in SLE. Also, there are signaling pathways for apoptosis including Fas-associated protein with death domain (FADD), tumor necrosis factor receptor-1 associated death domain (TRADD), FADD-like interleukin-113 converting enzyme (FLICE) which are important in apoptosis signaling. The bc1-2 family modulate apoptosis, and have been reported to be abnormal in human autoimmune disease. Soluble inhibitors of Fas apoptosis including a soluble form of Fas which lacks the transmembrane exon are elevated in SLE patients. Different genetic and environmental factors are proposed to interfere with apoptosis and clearance of inflammatory cells at several levels leading to the cellular defects of T cell dysfunction and B cell hyperactivity observed in patients with SLE. More... »

PAGES

181-212

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-3-0348-8741-0_11

DOI

http://dx.doi.org/10.1007/978-3-0348-8741-0_11

DIMENSIONS

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