Genetics of immunoglobulin-A vasculitis (Henoch-Schönlein purpura): An updated review López-Mejías, Raquel Castañeda, Santos Genre, Fernanda Remuzgo-Martínez, Sara Carmona López, Francisco David Llorca, Javier Blanco, Ricardo Martín, Javier González-Gay, Miguel A. Genetics Immunoglobulin-A vasculitis Henoch-Schönlein purpura We wish to specially thank Begoña Ubilla, Verónica Mijares, Virginia Portilla González, Rodrigo Ochoa, Marta Conde-Jaldón, Lourdes Ortiz-Fernández, Ana Márquez, Luis Sala-Icardo, Javier Rueda-Gotor, Belén Sevilla-Perez, Vanesa Calvo-Río, Natalia Palmou, María C. González-Vela, Francisca González Escribano, J. Gonzalo Ocejo-Vinyals, Javier Sánchez-Pérez, Antonio Navas Parejo, Diego de Argila, Maximiliano Aragües, Esteban Rubio, Manuel León Luque, Juan María Blanco-Madrigal, Eva Galíndez- Aguirregoikoa, José A. Miranda-Filloy, Trinitario Pina and Norberto Ortego-Centeno that have been involved in our studies. 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. 2018-07-02T10:18:43Z 2018-07-02T10:18:43Z 2018-01-17 info:eu-repo/semantics/article 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] http://hdl.handle.net/10481/51942 10.1016/j.autrev.2017.11.024 eng http://creativecommons.org/licenses/by-nc-nd/3.0/es/ info:eu-repo/semantics/openAccess Atribución-NoComercial-SinDerivadas 3.0 España Elsevier