{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T13:04:00Z","timestamp":1790773440506,"version":"4.1.0"},"reference-count":28,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2018,8,1]],"date-time":"2018-08-01T00:00:00Z","timestamp":1533081600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.fr","clinicalkey.com.au","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Autoimmunity Reviews"],"published-print":{"date-parts":[[2018,8]]},"DOI":"10.1016\/j.autrev.2018.02.012","type":"journal-article","created":{"date-parts":[[2018,6,8]],"date-time":"2018-06-08T02:01:23Z","timestamp":1528423283000},"page":"809-815","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":79,"title":["Autosomic dominant familial Beh\u00e7et disease and haploinsufficiency A20: A review of the literature"],"prefix":"10.1016","volume":"17","author":[{"given":"Florian","family":"Berteau","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9n\u00e9dicte","family":"Rouviere","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lien","family":"Delluc","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice","family":"Nau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rozenn","family":"Le Berre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Sarrabay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Touitou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"de Moreuil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.autrev.2018.02.012_bb0005","first-page":"471","article-title":"Beh\u00e7et's disease, hospital-based prevalence and manifestations in the Rotterdam area","volume":"73","author":"Kappen","year":"2015","journal-title":"Neth J Med"},{"key":"10.1016\/j.autrev.2018.02.012_bb0010","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1056\/NEJM199910213411707","article-title":"Beh\u00e7et's disease","volume":"341","author":"Sakane","year":"1999","journal-title":"N Engl J Med"},{"issue":"4","key":"10.1016\/j.autrev.2018.02.012_bb0015","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/j.autrev.2016.01.003","article-title":"Pulmonary artery aneurysms in Behcet's disease treated with anti-TNFalpha: a case series and review of the literature","volume":"15","author":"Chan","year":"2016","journal-title":"Autoimmun Rev"},{"issue":"3","key":"10.1016\/j.autrev.2018.02.012_bb0020","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.autrev.2015.11.011","article-title":"Behcet disease presenting with movement disorders and antibasal ganglia antibodies","volume":"15","author":"Rizzo","year":"2016","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.autrev.2018.02.012_bb0025","first-page":"607921","article-title":"Diagnosis\/classification criteria for Behcet's disease","author":"Davatchi","year":"2012","journal-title":"Pathol Res Int"},{"key":"10.1016\/j.autrev.2018.02.012_bb0030","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1136\/ard.38.2.118","article-title":"Beh\u00e7et's syndrome: a family study and the elucidation of a genetic role","volume":"38","author":"Berman","year":"1979","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.autrev.2018.02.012_bb0035","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1136\/ard.45.4.265","article-title":"Genetic analysis of families of patients with Beh\u00e7et's syndrome: data incompatible with autosomal recessive inheritance","volume":"45","author":"Bird Stewart","year":"1986","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.autrev.2018.02.012_bb0040","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1002\/ajmg.a.20136","article-title":"Identification of an autosomal recessive mode of inheritance in paediatric Beh\u00e7et's families by segregation analysis: Mendelian entity in paediatric Beh\u00e7et","volume":"122A","author":"Molinari","year":"2003","journal-title":"Am J Med Genet A"},{"key":"10.1016\/j.autrev.2018.02.012_bb0045","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1038\/ng.3459","article-title":"Loss-of-function mutations in TNFAIP3 leading to A20 haploinsufficiency cause an early-onset autoinflammatory disease","volume":"48","author":"Zhou","year":"2015","journal-title":"Nat Genet"},{"key":"10.1016\/j.autrev.2018.02.012_bb0050","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2017.00399","article-title":"NF-\u03baB pathway in autoinflammatory diseases: dysregulation of protein modifications by ubiquitin defines a new category of autoinflammatory diseases","volume":"8","author":"Aksentijevich","year":"2017","journal-title":"Front Immunol"},{"issue":"1","key":"10.1016\/j.autrev.2018.02.012_bb0055","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.it.2013.10.005","article-title":"A20 in inflammation and autoimmunity","volume":"35","author":"Catrysse","year":"2014","journal-title":"Trends Immunol"},{"key":"10.1016\/j.autrev.2018.02.012_bb0060","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2017-212403","article-title":"A20 haploinsufficiency (HA20): clinical phenotypes and disease course of patients with a newly recognised NF-kB-mediated autoinflammatory disease","author":"Aeschlimann","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.autrev.2018.02.012_bb0065","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.alit.2016.06.006","article-title":"A Japanese family case with juvenile onset Beh\u00e7et's disease caused by TNFAIP3 mutation","volume":"66","author":"Ohnishi","year":"2017","journal-title":"Allergol Int"},{"key":"10.1016\/j.autrev.2018.02.012_bb0070","doi-asserted-by":"crossref","first-page":"1914","DOI":"10.1016\/j.jaci.2016.09.038","article-title":"Haploinsufficiency of TNFAIP3 (A20) by germline mutation is involved in autoimmune lymphoproliferative syndrome","volume":"139","author":"Takagi","year":"2017","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.autrev.2018.02.012_bb0075","article-title":"Early-onset autoimmune disease due to a heterozygous loss-of-function mutation in TNFAIP3 (A20)","author":"Duncan","year":"2017","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.autrev.2018.02.012_bb0080","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2015-000223","article-title":"Novel heterozygous C243Y A20\/TNFAIP3 gene mutation is responsible for chronic inflammation in autosomal-dominant Beh\u00e7et9s disease","volume":"2","author":"Shigemura","year":"2016","journal-title":"RMD Open"},{"key":"10.1016\/j.autrev.2018.02.012_bb0085","doi-asserted-by":"crossref","first-page":"2154","DOI":"10.1097\/MIB.0000000000001269","article-title":"Phenotype and management of infantile-onset inflammatory bowel disease: experience from a tertiary care center in China","volume":"23","author":"Ye","year":"2017","journal-title":"Inflamm Bowel Dis"},{"key":"10.1016\/j.autrev.2018.02.012_bb0090","article-title":"Haploinsufficiency of A20 causes autoinflammatory and autoimmune disorders","author":"Kadowaki","year":"2017","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.autrev.2018.02.012_bb0095","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2018-213347","article-title":"Response to: A20 haploinsufficiency (HA20): clinical phenotypes and disease course of patients with a newly recognised NF-kB-mediated autoinflammatory disease","author":"Berteau","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.autrev.2018.02.012_bb0100","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1038\/nri3313","article-title":"A20: linking a complex regulator of ubiquitylation to immunity and human disease","volume":"12","author":"Ma","year":"2012","journal-title":"Nat Rev Immunol"},{"key":"10.1016\/j.autrev.2018.02.012_bb0105","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1007\/s12026-015-8677-6","article-title":"Roles of A20 in autoimmune diseases","volume":"64","author":"Zhang","year":"2016","journal-title":"Immunol Res"},{"key":"10.1016\/j.autrev.2018.02.012_bb0110","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1038\/ng.916","article-title":"A20 edits ubiquitin and autoimmune paradigms","volume":"43","author":"Martin","year":"2011","journal-title":"Nat Genet"},{"key":"10.1016\/j.autrev.2018.02.012_bb0115","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/j.rdc.2017.04.011","article-title":"Genomics, biology and human illness: advances in the monogenic autoinflammatory disease","volume":"43","author":"Oda","year":"2017","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.autrev.2018.02.012_bb0120","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1038\/nature13322","article-title":"Negative regulation of the NLRP3 inflammasome by A20 protects against arthritis","volume":"512","author":"Walle","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.autrev.2018.02.012_bb0125","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/j.immuni.2014.12.031","article-title":"A20 restricts ubiquitination of pro-interleukin-1\u03b2 protein complexes and suppresses NLRP3 inflammasome activity","volume":"42","author":"Duong","year":"2015","journal-title":"Immunity"},{"key":"10.1016\/j.autrev.2018.02.012_bb0130","doi-asserted-by":"crossref","first-page":"2350","DOI":"10.1126\/science.289.5488.2350","article-title":"Failure to regulate TNF-induced NF-\u03baB and cell death responses in A20-deficient mice","volume":"289","author":"Lee","year":"2000","journal-title":"Science"},{"key":"10.1016\/j.autrev.2018.02.012_bb0135","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1038\/nature07968","article-title":"Mutations of multiple genes cause deregulation of NF-\u03baB in diffuse large B-cell lymphoma","volume":"459","author":"Compagno","year":"2009","journal-title":"Nature"},{"key":"10.1016\/j.autrev.2018.02.012_bb0140","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1084\/jem.20090528","article-title":"TNFAIP3 (A20) is a tumor suppressor gene in Hodgkin lymphoma and primary mediastinal B cell lymphoma","volume":"206","author":"Schmitz","year":"2009","journal-title":"J Exp Med"}],"container-title":["Autoimmunity Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1568997218301393?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1568997218301393?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,7,20]],"date-time":"2018-07-20T04:07:31Z","timestamp":1532059651000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1568997218301393"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,8]]},"references-count":28,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2018,8]]}},"alternative-id":["S1568997218301393"],"URL":"https:\/\/doi.org\/10.1016\/j.autrev.2018.02.012","relation":{},"ISSN":["1568-9972"],"issn-type":[{"value":"1568-9972","type":"print"}],"subject":[],"published":{"date-parts":[[2018,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Autosomic dominant familial Beh\u00e7et disease and haploinsufficiency A20: A review of the literature","name":"articletitle","label":"Article Title"},{"value":"Autoimmunity Reviews","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.autrev.2018.02.012","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}