{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T18:25:02Z","timestamp":1782152702765,"version":"3.54.5"},"reference-count":47,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,4,29]],"date-time":"2020-04-29T00:00:00Z","timestamp":1588118400000},"content-version":"vor","delay-in-days":119,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001245","name":"Jeffrey Modell Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001245","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clin &amp;amp; Trans Imm"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Selective IgA deficiency (sIgAD) is the most common primary immunodeficiency in Western countries. Patients can suffer from recurrent infections and autoimmune diseases because of a largely unknown aetiology. To increase insights into the pathophysiology of the disease, we studied memory B and T cells and cytokine concentrations in peripheral blood.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We analysed 30 sIgAD patients (12 children, 18 adults) through detailed phenotyping of peripheral B\u2010cell, CD8<jats:sup>+<\/jats:sup> T\u2010cell and CD4<jats:sup>+<\/jats:sup> T\u2010cell subsets, sequence analysis of <jats:italic>IGA<\/jats:italic> and <jats:italic>IGG<\/jats:italic> transcripts, <jats:italic>in vitro<\/jats:italic> B\u2010cell activation and blood cytokine measurements.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All patients had significantly decreased numbers of T\u2010cell\u2010dependent (TD; CD27<jats:sup>+<\/jats:sup>) and T\u2010cell\u2010independent (TI; CD27<jats:sup>\u2212<\/jats:sup>) IgA memory B cells and increased CD21<jats:sup>low<\/jats:sup> B\u2010cell numbers. IgM<jats:sup>+<\/jats:sup>IgD<jats:sup>\u2212<\/jats:sup> memory B cells were decreased in children and normal in adult patients. <jats:italic>IGA<\/jats:italic> and <jats:italic>IGG<\/jats:italic> transcripts contained normal SHM levels. In sIgAD children, <jats:italic>IGA<\/jats:italic> transcripts more frequently used <jats:italic>IGA2<\/jats:italic> than controls (58.5% vs. 25.1%), but not in adult patients. B\u2010cell activation after <jats:italic>in vitro<\/jats:italic> stimulation was normal. However, adult sIgAD patients exhibited increased blood levels of TGF\u2010\u03b21, BAFF and APRIL, whereas they had decreased Th1 and Th17 cell numbers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Impaired IgA memory formation in sIgAD patients is not due to a B\u2010cell activation defect. Instead, decreased Th1 and Th17 cell numbers and high blood levels of BAFF, APRIL and TGF\u2010\u03b21 might reflect disturbed regulation of IgA responses <jats:italic>in vivo<\/jats:italic>.<\/jats:p><jats:p>These insights into B\u2010cell extrinsic immune defects suggest the need for a broader immunological focus on genomics and functional analyses to unravel the pathogenesis of sIgAD.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cti2.1130","type":"journal-article","created":{"date-parts":[[2020,4,30]],"date-time":"2020-04-30T04:15:45Z","timestamp":1588220145000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Defective formation of IgA memory B cells, Th1 and Th17 cells in symptomatic patients with selective IgA deficiency"],"prefix":"10.1002","volume":"9","author":[{"given":"Christina","family":"Grosserichter\u2010Wagener","sequence":"first","affiliation":[{"name":"Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Franco\u2010Gallego","sequence":"additional","affiliation":[{"name":"Grupo de Inmunodeficiencias Primarias Universidad de Antioquia UdeA Medell\u00edn Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatemeh","family":"Ahmadi","sequence":"additional","affiliation":[{"name":"Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcela","family":"Moncada\u2010V\u00e9lez","sequence":"additional","affiliation":[{"name":"Grupo de Inmunodeficiencias Primarias Universidad de Antioquia UdeA Medell\u00edn Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virgil ASH","family":"Dalm","sequence":"additional","affiliation":[{"name":"Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands"},{"name":"Department of Internal Medicine Erasmus MC University Medical Center Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica Lineth","family":"Rojas","sequence":"additional","affiliation":[{"name":"Grupo de Inmunodeficiencias Primarias Universidad de Antioquia UdeA Medell\u00edn Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julio C\u00e9sar","family":"Orrego","sequence":"additional","affiliation":[{"name":"Grupo de Inmunodeficiencias Primarias Universidad de Antioquia UdeA Medell\u00edn Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Correa Vargas","sequence":"additional","affiliation":[{"name":"Grupo de Inmunodeficiencias Primarias Universidad de Antioquia UdeA Medell\u00edn Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lennart","family":"Hammarstr\u00f6m","sequence":"additional","affiliation":[{"name":"Clinical Immunology Department of Laboratory Medicine Karolinska Institutet at Karolinska University Hospital Huddinge Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco WJ","family":"Schreurs","sequence":"additional","affiliation":[{"name":"Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willem A","family":"Dik","sequence":"additional","affiliation":[{"name":"Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P Martin","family":"van Hagen","sequence":"additional","affiliation":[{"name":"Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands"},{"name":"Department of Internal Medicine Erasmus MC University Medical Center Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Boon","sequence":"additional","affiliation":[{"name":"Bioceros B.V. 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