Genetics of immunoglobulin-A vasculitis (Henoch-Schönlein purpura): An updated review
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López-Mejías, Raquel; Castañeda, Santos; Genre, Fernanda; Remuzgo-Martínez, Sara; Carmona López, Francisco David; Llorca, Javier; Blanco, Ricardo; Martín, Javier; González-Gay, Miguel A.Editorial
Elsevier
Materia
Genetics Immunoglobulin-A vasculitis Henoch-Schönlein purpura
Date
2018-01-17Referencia bibliográfica
López-Mejías, Raquel; et.al. Genetics of immunoglobulin-A vasculitis (Henoch-Schönlein purpura): An updated review. Autoimmunity Reviews 17 (2018) 301–315 [http://hdl.handle.net/10481/51942]
Sponsorship
RL-Mis supported by the Miguel Servet I programme of the Spanish Ministry of Economy and Competitiveness through the grant CP16/ 00033. FG is recipient of a Sara Borrell postdoctoral fellowship from the “Instituto Carlos III de Salud” at the Spanish Ministry of Health (Spain) (CD15/00095). SR-M is supported by funds from the RETICS Program (RIER) (RD16/0012/0009). FDC is supported by the Ramón y Cajal programme of the Spanish Ministry of Economy and Competitiveness through the grant RYC-2014-16458.Abstract
Immunoglobulin-A vasculitis (IgAV) is classically a childhood small-sized blood vessel vasculitis with predominant
involvement of the skin. Gastrointestinal and joint manifestations are common in patients diagnosed
with this condition. Nephritis, which is more severe in adults, constitutes the most feared complication of this
vasculitis. The molecular bases underlying the origin of IgAV have not been completely elucidated. Nevertheless,
several pieces of evidence support the claim that genes play a crucial role in the pathogenesis of this disease. The
human leukocyte antigen (HLA) region is, until now, the main genetic factor associated with IgAV pathogenesis.
Besides a strong associationwith HLA class II alleles, specifically HLA-DRB1 alleles, HLA class I alleles also seemto
influence on the predisposition of this disease. Other gene polymorphisms located outside the HLA region, including
those coding cytokines, chemokines, adhesionmolecules aswell as those related to T-cells, aberrant glycosylation
of IgA1, nitric oxide production, neoangiogenesis, renin-angiotensin system and lipid, Pyrin and
homocysteine metabolism, may be implicated not only in the predisposition to IgAV but also in its severity. An
update of the current knowledge of the genetic component associated with the pathogenesis of IgAV is detailed
in this review.