PMID- 29353097 OWN - NLM STAT- MEDLINE DCOM- 20180521 LR - 20211204 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 17 IP - 3 DP - 2018 Mar TI - Genetics of immunoglobulin-A vasculitis (Henoch-Schonlein purpura): An updated review. PG - 301-315 LID - S1568-9972(18)30012-0 [pii] LID - 10.1016/j.autrev.2017.11.024 [doi] AB - 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 association with HLA class II alleles, specifically HLA-DRB1 alleles, HLA class I alleles also seem to influence on the predisposition of this disease. Other gene polymorphisms located outside the HLA region, including those coding cytokines, chemokines, adhesion molecules as well 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. CI - Copyright (c) 2018 The Author(s). Published by Elsevier B.V. All rights reserved. FAU - Lopez-Mejias, Raquel AU - Lopez-Mejias R AD - Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain. Electronic address: rlopezmejias78@gmail.com. FAU - Castaneda, Santos AU - Castaneda S AD - Division of Rheumatology, Hospital Universitario de la Princesa, IIS-IPrincesa, Madrid, Spain. FAU - Genre, Fernanda AU - Genre F AD - Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain. FAU - Remuzgo-Martinez, Sara AU - Remuzgo-Martinez S AD - Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain. FAU - Carmona, F David AU - Carmona FD AD - Instituto de Parasitologia y Biomedicina 'Lopez-Neyra', CSIC, PTS Granada, Granada, Spain; Departamento de Genetica e Instituto de Biotecnologia, Universidad de Granada, Granada, Spain. FAU - Llorca, Javier AU - Llorca J AD - Division of Epidemiology and Computational Biology, School of Medicine, University of Cantabria, CIBER Epidemiologia y Salud Publica (CIBERESP), IDIVAL, Santander, Spain. FAU - Blanco, Ricardo AU - Blanco R AD - Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain. FAU - Martin, Javier AU - Martin J AD - Instituto de Parasitologia y Biomedicina 'Lopez-Neyra', CSIC, PTS Granada, Granada, Spain. FAU - Gonzalez-Gay, Miguel A AU - Gonzalez-Gay MA AD - Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain; School of Medicine, University of Cantabria, Santander, Spain; Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. Electronic address: miguelaggay@hotmail.com. LA - eng PT - Journal Article PT - Review DEP - 20180117 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Immunoglobulin A) SB - IM MH - Humans MH - IgA Vasculitis/*genetics MH - Immunoglobulin A/*genetics/immunology MH - Polymorphism, Genetic/*genetics OTO - NOTNLM OT - Genetics OT - Henoch-Schonlein purpura OT - Immunoglobulin-A vasculitis EDAT- 2018/01/22 06:00 MHDA- 2018/05/22 06:00 CRDT- 2018/01/22 06:00 PHST- 2017/11/11 00:00 [received] PHST- 2017/11/16 00:00 [accepted] PHST- 2018/01/22 06:00 [pubmed] PHST- 2018/05/22 06:00 [medline] PHST- 2018/01/22 06:00 [entrez] AID - S1568-9972(18)30012-0 [pii] AID - 10.1016/j.autrev.2017.11.024 [doi] PST - ppublish SO - Autoimmun Rev. 2018 Mar;17(3):301-315. doi: 10.1016/j.autrev.2017.11.024. Epub 2018 Jan 17.