{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T19:51:15Z","timestamp":1787946675524,"version":"build-2784847793"},"reference-count":49,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2020,8,1]],"date-time":"2020-08-01T00:00:00Z","timestamp":1596240000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,8,1]],"date-time":"2020-08-01T00:00:00Z","timestamp":1596240000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,2,11]],"date-time":"2020-02-11T00:00:00Z","timestamp":1581379200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001034","name":"Australian Liver Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001034","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacionline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2020,8]]},"DOI":"10.1016\/j.jaci.2020.01.046","type":"journal-article","created":{"date-parts":[[2020,2,10]],"date-time":"2020-02-10T20:19:54Z","timestamp":1581365994000},"page":"417-428","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":34,"title":["Plasma protein profiling reflects TH1-driven immune dysregulation in common variable immunodeficiency"],"prefix":"10.1016","volume":"146","author":[{"given":"Jonas","family":"Hultberg","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Ernerudh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Larsson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c5sa","family":"Nilsdotter-Augustinsson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofia","family":"Nystr\u00f6m","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2020.01.046_bib1","doi-asserted-by":"crossref","first-page":"415","DOI":"10.3389\/fimmu.2014.00415","article-title":"Comparison of diagnostic criteria for common variable immunodeficiency disorder","volume":"5","author":"Ameratunga","year":"2014","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib2","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1006\/clim.1999.4725","article-title":"Common variable immunodeficiency: clinical and immunological features of 248 patients","volume":"92","author":"Cunningham-Rundles","year":"1999","journal-title":"Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib3","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1182\/blood-2007-06-091744","article-title":"The EUROclass trial: defining subgroups in common variable immunodeficiency","volume":"111","author":"Wehr","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib4","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1007\/s10875-017-0464-9","article-title":"International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee report on inborn errors of immunity","volume":"38","author":"Picard","year":"2018","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib5","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1016\/j.jaci.2011.02.039","article-title":"Genome-wide association identifies diverse causes of common variable immunodeficiency","volume":"127","author":"Orange","year":"2011","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib6","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1007\/s10875-015-0201-1","article-title":"Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 2015","volume":"35","author":"Picard","year":"2015","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib7","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1016\/j.jaci.2018.01.039","article-title":"Loss-of-function nuclear factor kappaB subunit 1 (NFKB1) variants are the most common monogenic cause of common variable immunodeficiency in Europeans","volume":"142","author":"Tuijnenburg","year":"2018","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib8","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/j.clim.2015.05.020","article-title":"Application of whole genome and RNA sequencing to investigate the genomic landscape of common variable immunodeficiency disorders","volume":"160","author":"van Schouwenburg","year":"2015","journal-title":"Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib9","doi-asserted-by":"crossref","first-page":"1650","DOI":"10.1182\/blood-2011-09-377945","article-title":"Morbidity and mortality in common variable immune deficiency over 4 decades","volume":"119","author":"Resnick","year":"2012","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib10","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1182\/blood-2007-11-124545","article-title":"Common variable immunodeficiency disorders: division into distinct clinical phenotypes","volume":"112","author":"Chapel","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib11","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1086\/606059","article-title":"Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect","volume":"49","author":"Malphettes","year":"2009","journal-title":"Clin Infect Dis"},{"key":"10.1016\/j.jaci.2020.01.046_bib12","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1007\/s10875-010-9424-3","article-title":"B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the disease","volume":"30","author":"Mouillot","year":"2010","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib13","doi-asserted-by":"crossref","first-page":"39710","DOI":"10.1038\/srep39710","article-title":"Common variable immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia","volume":"7","author":"Stuchly","year":"2017","journal-title":"Sci Rep"},{"key":"10.1016\/j.jaci.2020.01.046_bib14","doi-asserted-by":"crossref","first-page":"5026","DOI":"10.1182\/blood-2009-09-243071","article-title":"Complement receptor 2\/CD21- human naive B cells contain mostly autoreactive unresponsive clones","volume":"115","author":"Isnardi","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib15","doi-asserted-by":"crossref","first-page":"13451","DOI":"10.1073\/pnas.0901984106","article-title":"Circulating CD21low B cells in common variable immunodeficiency resemble tissue homing, innate-like B cells","volume":"106","author":"Rakhmanov","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2020.01.046_bib16","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.cellimm.2017.05.008","article-title":"Role of CD11c(+) T-bet(+) B cells in human health and disease","volume":"321","author":"Karnell","year":"2017","journal-title":"Cell Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib17","doi-asserted-by":"crossref","first-page":"1206","DOI":"10.1016\/j.jaci.2015.09.013","article-title":"Expansion of inflammatory innate lymphoid cells in patients with common variable immune deficiency","volume":"137","author":"Cols","year":"2016","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib18","doi-asserted-by":"crossref","first-page":"e74893","DOI":"10.1371\/journal.pone.0074893","article-title":"Interferon signature in the blood in inflammatory common variable immune deficiency","volume":"8","author":"Park","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2020.01.046_bib19","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1016\/j.jaci.2017.04.041","article-title":"The TH1 phenotype of follicular helper T cells indicates an IFN-gamma-associated immune dysregulation in patients with CD21low common variable immunodeficiency","volume":"141","author":"Unger","year":"2018","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib20","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/j.trsl.2018.11.001","article-title":"The contribution and perspectives of proteomics to uncover ovarian cancer tumor markers","volume":"206","author":"Carvalho","year":"2019","journal-title":"Transl Res"},{"key":"10.1016\/j.jaci.2020.01.046_bib21","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1007\/s40620-018-0556-5","article-title":"Circulating proteins as predictors of cardiovascular mortality in end-stage renal disease","volume":"32","author":"Feldreich","year":"2019","journal-title":"J Nephrol"},{"key":"10.1016\/j.jaci.2020.01.046_bib22","doi-asserted-by":"crossref","first-page":"2755","DOI":"10.1038\/s41467-018-05044-4","article-title":"Multi-omics monitoring of drug response in rheumatoid arthritis in pursuit of molecular remission","volume":"9","author":"Tasaki","year":"2018","journal-title":"Nat Commun"},{"key":"10.1016\/j.jaci.2020.01.046_bib23","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.ebiom.2018.08.039","article-title":"Assessing statin effects on cardiovascular pathways in HIV using a novel proteomics approach: Analysis of data from INTREPID, a randomized controlled trial","volume":"35","author":"Toribio","year":"2018","journal-title":"EBioMedicine"},{"key":"10.1016\/j.jaci.2020.01.046_bib24","doi-asserted-by":"crossref","first-page":"e95192","DOI":"10.1371\/journal.pone.0095192","article-title":"Homogenous 96-plex PEA immunoassay exhibiting high sensitivity, specificity, and excellent scalability","volume":"9","author":"Assarsson","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2020.01.046_bib25","doi-asserted-by":"crossref","first-page":"e102","DOI":"10.1093\/nar\/gkr424","article-title":"Homogeneous antibody-based proximity extension assays provide sensitive and specific detection of low-abundant proteins in human blood","volume":"39","author":"Lundberg","year":"2011","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2020.01.046_bib26","doi-asserted-by":"crossref","first-page":"1544","DOI":"10.1182\/blood.V99.5.1544","article-title":"Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease","volume":"99","author":"Warnatz","year":"2002","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib27","doi-asserted-by":"crossref","first-page":"W147","DOI":"10.1093\/nar\/gkw419","article-title":"Heatmapper: web-enabled heat mapping for all","volume":"44","author":"Babicki","year":"2016","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2020.01.046_bib28","doi-asserted-by":"crossref","first-page":"W486","DOI":"10.1093\/nar\/gky310","article-title":"MetaboAnalyst 4.0: towards more transparent and integrative metabolomics analysis","volume":"46","author":"Chong","year":"2018","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2020.01.046_bib29","doi-asserted-by":"crossref","first-page":"3932","DOI":"10.4049\/jimmunol.178.6.3932","article-title":"Unravelling the complexity of T cell abnormalities in common variable immunodeficiency","volume":"178","author":"Giovannetti","year":"2007","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib30","doi-asserted-by":"crossref","DOI":"10.1016\/j.jaip.2019.02.004","article-title":"The European Society for Immunodeficiencies (ESID) registry working definitions for the clinical diagnosis of inborn errors of immunity","author":"Seidel","year":"2019","journal-title":"J Allergy Clin Immunol Pract"},{"key":"10.1016\/j.jaci.2020.01.046_bib31","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1007\/s10875-014-0099-z","article-title":"Altered serum cytokine signature in common variable immunodeficiency","volume":"34","author":"Hel","year":"2014","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib32","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1084\/jem.20140039","article-title":"Exhaustion of bacteria-specific CD4 T cells and microbial translocation in common variable immunodeficiency disorders","volume":"211","author":"Perreau","year":"2014","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2020.01.046_bib33","doi-asserted-by":"crossref","first-page":"23","DOI":"10.4049\/jimmunol.1000832","article-title":"Cutting edge: FcR-like 6 is an MHC class II receptor","volume":"185","author":"Schreeder","year":"2010","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib34","doi-asserted-by":"crossref","first-page":"3786","DOI":"10.1182\/blood-2006-06-030023","article-title":"FcRL6, a new ITIM-bearing receptor on cytolytic cells, is broadly expressed by lymphocytes following HIV-1 infection","volume":"109","author":"Wilson","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib35","doi-asserted-by":"crossref","first-page":"17775","DOI":"10.1038\/s41598-017-18195-z","article-title":"Whole transcriptome profiling reveals major cell types in the cellular immune response against acute and chronic active Epstein-Barr virus infection","volume":"7","author":"Zhong","year":"2017","journal-title":"Sci Rep"},{"key":"10.1016\/j.jaci.2020.01.046_bib36","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1007\/978-0-387-89520-8_8","article-title":"4-1BB as a therapeutic target for human disease","volume":"647","author":"Lee","year":"2009","journal-title":"Adv Exp Med Biol"},{"key":"10.1016\/j.jaci.2020.01.046_bib37","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1182\/blood-2017-07-741025","article-title":"The immunobiology of CD27 and OX40 and their potential as targets for cancer immunotherapy","volume":"131","author":"Buchan","year":"2018","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2020.01.046_bib38","doi-asserted-by":"crossref","first-page":"1758","DOI":"10.1038\/s41467-018-03750-7","article-title":"IL-21 drives expansion and plasma cell differentiation of autoreactive CD11c(hi)T-bet(+) B cells in SLE","volume":"9","author":"Wang","year":"2018","journal-title":"Nat Commun"},{"key":"10.1016\/j.jaci.2020.01.046_bib39","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.41641","article-title":"IFNgamma induces epigenetic programming of human T-bet(hi) B cells and promotes TLR7\/8 and IL-21 induced differentiation","volume":"8","author":"Zumaquero","year":"2019","journal-title":"Elife"},{"key":"10.1016\/j.jaci.2020.01.046_bib40","doi-asserted-by":"crossref","first-page":"1730","DOI":"10.1002\/art.34330","article-title":"A phase II, randomized, double-blind, placebo-controlled study evaluating the efficacy and safety of MDX-1100, a fully human anti-CXCL10 monoclonal antibody, in combination with methotrexate in patients with rheumatoid arthritis","volume":"64","author":"Yellin","year":"2012","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.jaci.2020.01.046_bib41","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1016\/j.ajhg.2015.07.008","article-title":"Haploinsufficiency of the NF-kappaB1 subunit p50 in common variable immunodeficiency","volume":"97","author":"Fliegauf","year":"2015","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.jaci.2020.01.046_bib42","doi-asserted-by":"crossref","first-page":"297","DOI":"10.3389\/fimmu.2019.00297","article-title":"Clinical and immunological phenotype of patients with primary immunodeficiency due to damaging mutations in NFKB2","volume":"10","author":"Klemann","year":"2019","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib43","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1038\/35018581","article-title":"A chemokine-driven positive feedback loop organizes lymphoid follicles","volume":"406","author":"Ansel","year":"2000","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2020.01.046_bib44","doi-asserted-by":"crossref","first-page":"3734","DOI":"10.4049\/jimmunol.1103246","article-title":"Bcl6 and Maf cooperate to instruct human follicular helper CD4 T cell differentiation","volume":"188","author":"Kroenke","year":"2012","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib45","doi-asserted-by":"crossref","first-page":"2497","DOI":"10.1084\/jem.20111449","article-title":"Follicular dendritic cells help establish follicle identity and promote B cell retention in germinal centers","volume":"208","author":"Wang","year":"2011","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2020.01.046_bib46","doi-asserted-by":"crossref","first-page":"2702","DOI":"10.1073\/pnas.1520112113","article-title":"CXCL13 is a plasma biomarker of germinal center activity","volume":"113","author":"Havenar-Daughton","year":"2016","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2020.01.046_bib47","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/j.jaci.2018.06.012","article-title":"Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses","volume":"143","author":"Romberg","year":"2019","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib48","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.jaci.2013.06.047","article-title":"High-content cytometry and transcriptomic biomarker profiling of human B-cell activation","volume":"133","author":"Hennig","year":"2014","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2020.01.046_bib49","doi-asserted-by":"crossref","DOI":"10.1126\/scisignal.aan3589","article-title":"Interleukin-36 cytokines may overcome microbial immune evasion strategies that inhibit interleukin-1 family signaling","volume":"10","author":"Jensen","year":"2017","journal-title":"Sci Signal"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674920301895?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674920301895?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T09:26:10Z","timestamp":1758792370000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674920301895"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8]]},"references-count":49,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2020,8]]}},"alternative-id":["S0091674920301895"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2020.01.046","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2020,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Plasma protein profiling reflects TH1-driven immune dysregulation in common variable immunodeficiency","name":"articletitle","label":"Article Title"},{"value":"Journal of Allergy and Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaci.2020.01.046","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 The Authors. Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology.","name":"copyright","label":"Copyright"}]}}