{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T05:38:50Z","timestamp":1787636330136,"version":"build-2736575974"},"reference-count":27,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,5,30]],"date-time":"2017-05-30T00:00:00Z","timestamp":1496102400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Scand J Immunol"],"published-print":{"date-parts":[[2017,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    In this study, we identified all adults living in Denmark diagnosed with common variable immunodeficiency (\n                    <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\n                    ) and characterized them according to clinical presentation and\n                    <jats:styled-content style=\"fixed-case\">EURO<\/jats:styled-content>\n                    class classification. Using a retrospective, cross\u2010sectional design, possible\n                    <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\n                    patients were identified in the Danish National Patient Register and Centers in Denmark treating patients with primary immunodeficiencies. The\n                    <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\n                    diagnosis was verified by review of medical records. One\u2010hundred\u2010seventy\u2010nine adults with\n                    <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\n                    were identified. This corresponds to a prevalence of 1:26,000. The median age at onset of symptoms was 29\u00a0years with no sex difference. The median age at diagnosis was 40\u00a0years. Males were diagnosed earlier with a peak in the fourth decade of life, whereas females were diagnosed later with a peak in the sixth decade. The median diagnostic delay was seven years. Recurrent sinopulmonary infections were seen in 92.7% of the patients. The prevalence of non\u2010infectious complications was similar to that of previously reported cohorts: bronchiectasis (35.8%), splenomegaly (22.4%), lymphadenopathy (26.3%), granulomatous inflammation (3.9%) and idiopathic thrombocytopenic purpura (14.5%). Non\u2010infectious complications were strongly associated with B cell phenotype, with all having a reduced number of isotype\u2010switched memory B cells. One\u2010hundred\u2010seventy (95%) were treated with immunoglobulin replacement therapy, primarily administered subcutaneously. According to international guidelines, diagnostic evaluation was inadequate in most cases. This study emphasizes the need for improved diagnostic criteria and more awareness of\n                    <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\n                    as a differential diagnosis. Diagnosis and management of\n                    <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\n                    patients is a challenge requiring specialists with experience in the field of\n                    <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>\n                    .\n                  <\/jats:p>","DOI":"10.1111\/sji.12551","type":"journal-article","created":{"date-parts":[[2017,4,4]],"date-time":"2017-04-04T18:54:15Z","timestamp":1491332055000},"page":"450-461","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":66,"title":["Identification and Characterization of a Nationwide Danish Adult Common Variable Immunodeficiency Cohort"],"prefix":"10.1111","volume":"85","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8333-2777","authenticated-orcid":false,"given":"L.","family":"Westh","sequence":"first","affiliation":[{"name":"Department of Infectious Diseases Aarhus University Hospital Skejby Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. H.","family":"Mogensen","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Aarhus University Hospital Skejby Aarhus Denmark"},{"name":"International Center of Immunodeficiency Diseases Aarhus University Hospital Skejby Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. S.","family":"Dalgaard","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Aarhus University Hospital Skejby Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. M.","family":"Bernth Jensen","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology Aarhus University Hospital Skejby Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Katzenstein","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Copenhagen University Hospital, Rigshospitalet Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.\u2010B. E.","family":"Hansen","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Copenhagen University Hospital, Hvidovre Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O. D.","family":"Larsen","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Odense University Hospital Odense Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Terpling","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Aalborg University Hospital Aalborg Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. L.","family":"Nielsen","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases North Sealand Hospital Hilleroed Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. S.","family":"Larsen","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases Aarhus University Hospital Skejby Aarhus Denmark"},{"name":"International Center of Immunodeficiency Diseases Aarhus University Hospital Skejby Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,5,30]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-01-254417"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4799"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-12-385991-4.00002-7"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4725"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-124545"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2013.12.1077"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.12178"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1067\/mai.2002.124999"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.5.1544"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1025373601374"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-06-091744"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2015.11.003"},{"key":"e_1_2_6_14_1","first-page":"263","article-title":"The Danish National Hospital Register. 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