{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T08:15:49Z","timestamp":1789460149427,"version":"build-2803163510"},"reference-count":40,"publisher":"Korean Association of Internal Medicine","issue":"5","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003710","name":"Korea Health Industry Development Institute","doi-asserted-by":"publisher","award":["RS-2024-00439277"],"award-info":[{"award-number":["RS-2024-00439277"]}],"id":[{"id":"10.13039\/501100003710","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003625","name":"Ministry of Health and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003625","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["kjim.org"],"crossmark-restriction":true},"short-container-title":["Korean J Intern Med"],"published-print":{"date-parts":[[2026,9,1]]},"abstract":"<jats:p>Background\/Aims: Primary antibody deficiency (PAD) in adults is increasingly recognized, but remains underdiagnosed and undertreated. We described the clinical and immunologic features of adult patients with PAD according to real-world exposure to intravenous immunoglobulin replacement therapy (IVIGRT) and explored whether the Ajou-PAD score reflects treatment decisions in routine clinical practice.Methods: We conducted a retrospective cohort study at Ajou University Hospital (January 1, 2020\u2013November 18, 2024), screening adults (age \u2265 18 yr) diagnosed with PAD, and excluding those with IVIGRT exposure before diagnosis. After 1:1 propensity score matching by age and sex, clinical and laboratory findings between the IVIGRT-exposed and -unexposed groups were compared. The discriminatory performance of the Ajou-PAD score, a composite index integrating clinical and immunologic parameters, was evaluated for IVIGRT exposure using receiver operating characteristic analysis.Results: The final matched cohort included 602 patients (301 per group). Immunoglobulin G subclass 3 deficiency (IgG3SCD) was the most prevalent phenotype and more frequent in the IVIGRT-exposed group than in the IVIGRT-unexposed group. The Ajou-PAD score showed modest discriminatory performance for IVIGRT exposure (AUC = 0.61; 95% CI, 0.56\u20130.65).Conclusions: In Korean adults with PAD, IgG3SCD was the most prevalent phenotype and was more common among patients receiving IVIGRT in routine clinical practice. The Ajou-PAD score showed modest discriminatory performance for IVIGRT exposure and should be considered an exploratory adjunct rather than a standalone tool for treatment decision-making.<\/jats:p>","DOI":"10.3904\/kjim.2026.072","type":"journal-article","created":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T02:15:56Z","timestamp":1788747356000},"page":"940-951","update-policy":"https:\/\/doi.org\/10.3904\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical and immunologic features of adult primary antibody deficiency according to IVIGRT exposure"],"prefix":"10.3904","volume":"41","author":[{"given":"Jung-Eun","family":"Han","sequence":"first","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyun young","family":"Lee","sequence":"additional","affiliation":[{"name":"Clinical Trial Center, Ajou University Medical Center, Suwon, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haeng Jun","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoo Seob","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yeong-Min","family":"Ye","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2614-0303","authenticated-orcid":true,"given":"Hae-Sim","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2270","reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2023.1245718","type":"journal-article"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01289-3","type":"journal-article"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2004.01.761","type":"journal-article"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-012-9751-7","type":"journal-article"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.2500\/aap.2024.45.240052","type":"journal-article"},{"key":"ref6","first-page":"116","type":"journal-article","volume-title":"Clinical picture and treatment of 2212 patients with common variable immunodeficiency","author":"Gathmann","year":"2014"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2021.13.2.271","type":"journal-article"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2017.9.6.526","type":"journal-article"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-124545","type":"journal-article"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-007-9075-1","type":"journal-article"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-377945","type":"journal-article"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2015.07.025","type":"journal-article"},{"key":"ref13","first-page":"1186","type":"journal-article","volume-title":"Practice parameter for the diagnosis and management of primary immunodeficiency","author":"Bonilla","year":"2015"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-025-01970-3","type":"journal-article"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-022-08942-0","type":"journal-article"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2023.15.5.562","type":"journal-article"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1007\/s15010-012-0323-9","type":"journal-article"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2006.01.015","type":"journal-article"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2016.09.023","type":"journal-article"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2013.01.036","type":"journal-article"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1111\/cei.13526","type":"journal-article"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1016\/j.jclinepi.2007.11.008","type":"journal-article"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2021.13.4.671","type":"journal-article"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1093\/biomet\/70.1.41","type":"journal-article"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.1080\/00273171.2011.568786","type":"journal-article"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.3390\/ijerph17186551","type":"journal-article"},{"key":"ref27","first-page":"99","type":"journal-article","volume-title":"","author":"Dupas","year":"2013"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(1950)3:1<32::aid-cncr2820030106>3.0.co;2-3","type":"journal-article"},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.3904\/kjim.2018.413","type":"journal-article"},{"key":"ref30","doi-asserted-by":"publisher","DOI":"10.3346\/jkms.2010.25.6.824","type":"journal-article"},{"key":"ref31","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1552513","type":"journal-article"},{"key":"ref32","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2024.16.5.462","type":"journal-article"},{"key":"ref33","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2024.16.1.22","type":"journal-article"},{"key":"ref34","doi-asserted-by":"publisher","DOI":"10.4168\/aair.2025.17.1.8","type":"journal-article"},{"key":"ref35","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2026.1787739","type":"journal-article"},{"key":"ref36","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2010.06.012","type":"journal-article"},{"key":"ref37","doi-asserted-by":"publisher","DOI":"10.1586\/eci.10.9","type":"journal-article"},{"key":"ref38","doi-asserted-by":"publisher","DOI":"10.1016\/j.iac.2008.01.008","type":"journal-article"},{"key":"ref39","doi-asserted-by":"publisher","DOI":"10.1080\/1750743x.2024.2382074","type":"journal-article"},{"key":"ref40","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2017.12.980","type":"journal-article"}],"container-title":["The Korean Journal of Internal Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/kjim.org\/upload\/kjim-2026-072.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/kjim.org\/journal\/view.php?doi=10.3904\/kjim.2026.072","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/kjim.org\/upload\/kjim-2026-072.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T07:59:34Z","timestamp":1789459174000},"score":1,"resource":{"primary":{"URL":"http:\/\/kjim.org\/journal\/view.php?doi=10.3904\/kjim.2026.072"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,1]]},"references-count":40,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,9]]}},"URL":"https:\/\/doi.org\/10.3904\/kjim.2026.072","relation":{},"ISSN":["1226-3303","2005-6648"],"issn-type":[{"value":"1226-3303","type":"print"},{"value":"2005-6648","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,1]]},"assertion":[{"value":"2026-02-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-28","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-01","order":2,"name":"published","label":"Published online","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-01","order":3,"name":"published","label":"Print publication","group":{"name":"publication_history","label":"Publication History"}}]}}