{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T07:22:51Z","timestamp":1782976971899,"version":"3.54.5"},"reference-count":43,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2018,10,1]],"date-time":"2018-10-01T00:00:00Z","timestamp":1538352000000},"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.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Seminars in Arthritis and Rheumatism"],"published-print":{"date-parts":[[2018,10]]},"DOI":"10.1016\/j.semarthrit.2018.02.013","type":"journal-article","created":{"date-parts":[[2018,2,23]],"date-time":"2018-02-23T05:09:51Z","timestamp":1519362591000},"page":"318-326","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":39,"title":["Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency"],"prefix":"10.1016","volume":"48","author":[{"given":"Maria J.","family":"Gutierrez","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen E.","family":"Sullivan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramsay","family":"Fuleihan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"Clifton O.","family":"Bingham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.semarthrit.2018.02.013_bib1","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1007\/s10875-007-9103-1","article-title":"Population prevalence of diagnosed primary immunodeficiency diseases in the United States","volume":"27","author":"Boyle","year":"2007","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib2","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1586\/1744666X.5.2.167","article-title":"Common variable immunodeficiency: a multifaceted and puzzling disorder","volume":"5","author":"Bergbreiter","year":"2009","journal-title":"Expert Rev Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib3","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/j.autrev.2012.10.017","article-title":"Chipping away at a mountain: genomic studies in common variable immunodeficiency","volume":"12","author":"Keller","year":"2013","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib4","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.semarthrit.2018.02.013_bib5","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","author":"Park","year":"2013","journal-title":"PLoS One 8"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib6","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.clim.2008.02.013","article-title":"Memory B cells in common variable immunodeficiency: clinical associations and sex differences","volume":"128","author":"Sanchez-Ramon","year":"2008","journal-title":"Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib7","first-page":"323","article-title":"Autoimmune phenotype in patients with common variable immunodeficiency","volume":"23","author":"Abolhassani","year":"2013","journal-title":"J Investig Allergol Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib8","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.jaut.2010.10.002","article-title":"Autoimmunity in common variable immunodeficiency: correlation with lymphocyte phenotype in the French DEFI study","volume":"36","author":"Boileau","year":"2011","journal-title":"J Autoimmun"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib9","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.semarthrit.2018.02.013_bib10","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.semarthrit.2018.02.013_bib11","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1007\/s11882-016-0597-6","article-title":"Autoimmune and lymphoproliferative complications of common variable immunodeficiency","volume":"16","author":"Maglione","year":"2016","journal-title":"Curr Allergy Asthma Rep"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib12","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.jaci.2013.12.1077","article-title":"Clinical picture and treatment of 2212 patients with common variable immunodeficiency","volume":"134","author":"Gathmann","year":"2014","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib13","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1007\/s11882-009-0051-0","article-title":"Autoimmunity in common variable immunodeficiency","volume":"9","author":"Agarwal","year":"2009","journal-title":"Curr Allergy Asthma Rep"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib14","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1038\/nature12873","article-title":"Genetics of rheumatoid arthritis contributes to biology and drug discovery","volume":"506","author":"Okada","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib15","doi-asserted-by":"crossref","first-page":"8442","DOI":"10.1038\/ncomms9442","article-title":"Genetic sharing and heritability of paediatric age of onset autoimmune diseases","volume":"6","author":"Li","year":"2015","journal-title":"Nat Commun"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib16","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.patbio.2014.04.002","article-title":"Common variable immunodeficiency (CVID): clinical and immunological features of 29 Algerian patients","volume":"62","author":"Tahiat","year":"2014","journal-title":"Pathol Biol (Paris)"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib17","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.aller.2013.01.007","article-title":"Clinical and immunological features of common variable immunodeficiency in Mexican patients","volume":"42","author":"Ramirez-Vargas","year":"2014","journal-title":"Allergol Immunopathol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib18","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/S0973-3698(12)60029-6","article-title":"Rheumatic manifestations of primary immunodeficiencies in children","volume":"7","author":"Agarwal","year":"2012","journal-title":"Indian J Rheumatol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib19","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1007\/s10875-014-0028-1","article-title":"USIDNET: a strategy to build a community of clinical immunologists","volume":"34","author":"Sullivan","year":"2014","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib20","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1056\/NEJMoa1512234","article-title":"Loss of B cells in patients with heterozygous mutations in IKAROS","volume":"374","author":"Kuehn","year":"2016","journal-title":"N Engl J Med"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib21","unstructured":"I. D. Foundation. (2014), vol. 2016."},{"key":"10.1016\/j.semarthrit.2018.02.013_bib22","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1080\/15459620490462797","article-title":"Imputation of data values that are less than a detection limit","volume":"1","author":"Succop","year":"2004","journal-title":"J Occup Environ Hyg"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib23","first-page":"115","article-title":"Risk for rheumatic disease in relation to ethnicity and admixture","volume":"2","author":"Molokhia","year":"2000","journal-title":"i"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib24","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.rdc.2014.04.001","article-title":"Impact of race and ethnicity in the course and outcome of systemic lupus erythematosus","volume":"40","author":"Gonzalez","year":"2014","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib25","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1038\/ni0901-777","article-title":"Sex differences in autoimmune disease","volume":"2","author":"Whitacre","year":"2001","journal-title":"Nat Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib26","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.jaci.2015.11.004","article-title":"The crossroads of autoimmunity and immunodeficiency: lessons from polygenic traits and monogenic defects","volume":"137","author":"Grimbacher","year":"2016","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib27","doi-asserted-by":"crossref","first-page":"1931","DOI":"10.1172\/JCI107377","article-title":"Abnormalities in the distribution of serum immunoglobulin concentrations in juvenile rheumatoid arthritis","volume":"52","author":"Cassidy","year":"1973","journal-title":"J Clin Invest"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib28","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1007\/s11102-015-0699-5","article-title":"Prevalence of autoimmune disease in patients with prolactinomas and non-functioning pituitary adenomas","volume":"19","author":"Larouche","year":"2016","journal-title":"Pituitary"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib29","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1016\/0049-0172(93)80073-O","article-title":"Hypogammaglobulinemia and rheumatic disease","volume":"22","author":"Lee","year":"1993","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib30","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1097\/00003086-199010000-00001","article-title":"Immunologic abnormalities of juvenile arthritis","author":"Miller","year":"1990","journal-title":"Clin Orthop Relat Res"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib31","doi-asserted-by":"crossref","first-page":"R114","DOI":"10.1186\/ar2773","article-title":"Markers of B-lymphocyte activation are elevated in patients with early rheumatoid arthritis and correlated with disease activity in the ESPOIR cohort","volume":"11","author":"Gottenberg","year":"2009","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib32","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1093\/rheumatology\/keq338","article-title":"Cytokine pattern in very early rheumatoid arthritis favours B-cell activation and survival","volume":"50","author":"Moura","year":"2011","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib33","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1038\/nature20810","article-title":"Pathologically expanded peripheral T helper cell subset drives B cells in rheumatoid arthritis","volume":"542","author":"Rao","year":"2017","journal-title":"Nature"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib34","doi-asserted-by":"crossref","first-page":"3692","DOI":"10.1002\/art.30599","article-title":"Blood memory B cells are disturbed and predict the response to rituximab in patients with rheumatoid arthritis","volume":"63","author":"Sellam","year":"2011","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib35","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1111\/j.1749-6632.1965.tb19007.x","article-title":"The frequent occurrence of hypergammaglobulinemia and multiple tissue antibodies in Sjogren\u2019s syndrome","volume":"124","author":"Bunim","year":"1965","journal-title":"Ann NY Acad Sci"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib36","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1136\/ard.51.11.1185","article-title":"Hypergammaglobulinaemia and autoimmune rheumatic diseases","volume":"51","author":"Ehrenstein","year":"1992","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib37","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1007\/s10875-016-0245-x","article-title":"Deficiency of adenosine deaminase 2 causes antibody deficiency","volume":"36","author":"Schepp","year":"2016","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib38","doi-asserted-by":"crossref","first-page":"4135","DOI":"10.1172\/JCI80477","article-title":"Broad-spectrum antibodies against self-antigens and cytokines in RAG deficiency","volume":"125","author":"Walter","year":"2015","journal-title":"J Clin Invest"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib39","doi-asserted-by":"crossref","first-page":"3154","DOI":"10.1182\/blood-2015-11-679902","article-title":"Heterozygous STAT1 gain-of-function mutations underlie an unexpectedly broad clinical phenotype","volume":"127","author":"Toubiana","year":"2016","journal-title":"Blood"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib40","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1586\/eci.09.43","article-title":"Role of B cells in common variable immune deficiency","volume":"5","author":"Ahn","year":"2009","journal-title":"Expert Rev Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib41","first-page":"321","article-title":"Analysis of class-switched memory B cells in patients with common variable immunodeficiency and its clinical implications","volume":"17","author":"Vodjgani","year":"2007","journal-title":"J Investig Allergol Clin Immunol"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib42","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1177\/0961203316643172","article-title":"B-cell subsets, signaling and their roles in secretion of autoantibodies","volume":"25","author":"Iwata","year":"2016","journal-title":"Lupus"},{"key":"10.1016\/j.semarthrit.2018.02.013_bib43","doi-asserted-by":"crossref","first-page":"1629","DOI":"10.1016\/j.jaci.2016.11.022","article-title":"Ruxolitinib reverses dysregulated T helper cell responses and controls autoimmunity caused by a novel signal transducer and activator of transcription 1 (STAT1) gain-of-function mutation","volume":"139","author":"Weinacht","year":"2017","journal-title":"J Allergy Clin Immunol"}],"container-title":["Seminars in Arthritis and Rheumatism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0049017217306546?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0049017217306546?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,8,14]],"date-time":"2022-08-14T20:00:30Z","timestamp":1660507230000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0049017217306546"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10]]},"references-count":43,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2018,10]]}},"alternative-id":["S0049017217306546"],"URL":"https:\/\/doi.org\/10.1016\/j.semarthrit.2018.02.013","relation":{},"ISSN":["0049-0172"],"issn-type":[{"value":"0049-0172","type":"print"}],"subject":[],"published":{"date-parts":[[2018,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency","name":"articletitle","label":"Article Title"},{"value":"Seminars in Arthritis and Rheumatism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.semarthrit.2018.02.013","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}