{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,9]],"date-time":"2025-12-09T12:41:00Z","timestamp":1765284060476,"version":"3.46.0"},"reference-count":10,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2025,9,29]],"date-time":"2025-09-29T00:00:00Z","timestamp":1759104000000},"content-version":"vor","delay-in-days":271,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100014013","name":"United Kingdom Research and Innovation","doi-asserted-by":"crossref","award":["MR\/W002663\/1"],"award-info":[{"award-number":["MR\/W002663\/1"]}],"id":[{"id":"10.13039\/100014013","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,1,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>B-cell maturation antigen (BCMA) is a B cell surface receptor that regulates B cell activation, proliferation and survival. BCMA can be cleaved from the cell surface, producing soluble BCMA (sBCMA), which has been studied as a disease biomarker in systemic lupus erythematosus, multiple sclerosis and multiple myeloma. Reduced sBCMA concentrations have been associated with the severity of different primary antibody deficiencies. We explored the relationship between sBCMA concentrations, humoral immune responses to SARS-CoV-2 vaccination and disease complications in 107 individuals with primary (PAD) and secondary antibody deficiency (SAD) enrolled in the COVID-19 in Antibody Deficiency (COV-AD) study. Serum sBCMA concentrations were significantly reduced in PAD compared to healthy controls and asymptomatic selective IgA deficiency. Individuals with X- linked agammaglobulinemia and common variable immunodeficiency (CVID) demonstrated the lowest serum concentrations of sBCMA. sBCMA concentrations in SAD were highly variable. Amongst individuals with CVID, peripheral blood CD19 count, but not sBCMA concentrations discriminated SARS-CoV-2 vaccine responders. sBCMA was significantly lower in individuals with CVID and bronchiectasis and outperformed serum IgA and IgM concentrations in discriminating this subgroup. sBCMA was not associated with any other complication of CVID. Our data highlights the potential of sBCMA as biomarker to support the assessment of antibody deficiency. In PAD, sBCMA may contribute to the risk stratification of disease severity and identify those at risk of bronchiectasis. In SAD, it may identify subgroups that would benefit from intensive monitoring and therapy.<\/jats:p>","DOI":"10.1093\/cei\/uxaf065","type":"journal-article","created":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T12:09:25Z","timestamp":1758888565000},"source":"Crossref","is-referenced-by-count":0,"title":["Serum sBCMA in primary and secondary antibody deficiency"],"prefix":"10.1093","volume":"219","author":[{"given":"Danai","family":"Bagkou Dimakou","sequence":"first","affiliation":[{"name":"Clinical Immunology Services, School of Infection, Inflammation and Immunology, College of Medicine and Health, University of Birmingham , Birmingham ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-9408-3774","authenticated-orcid":false,"given":"Nicholas E","family":"Peters","sequence":"additional","affiliation":[{"name":"Clinical Immunology Services, School of Infection, Inflammation and Immunology, College of Medicine and Health, University of Birmingham , Birmingham ,","place":["UK"]},{"name":"Department of Clinical Immunology, University Hospitals Birmingham NHS Foundation Trust , Birmingham ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siobhan O","family":"Burns","sequence":"additional","affiliation":[{"name":"Institute of Immunity and Transplantation, University College London , London ,","place":["UK"]},{"name":"Department of Immunology, Royal Free London NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alex G","family":"Richter","sequence":"additional","affiliation":[{"name":"Clinical Immunology Services, School of Infection, Inflammation and Immunology, College of Medicine and Health, University of Birmingham , Birmingham ,","place":["UK"]},{"name":"Department of Clinical Immunology, University Hospitals Birmingham NHS Foundation Trust , Birmingham ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5345-2156","authenticated-orcid":false,"given":"Adrian M","family":"Shields","sequence":"additional","affiliation":[{"name":"Clinical Immunology Services, School of Infection, Inflammation and Immunology, College of Medicine and Health, University of Birmingham , Birmingham ,","place":["UK"]},{"name":"Department of Clinical Immunology, University Hospitals Birmingham NHS Foundation Trust , Birmingham ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,9,29]]},"reference":[{"key":"2025120907362236100_uxaf065-B1","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/j.cytogfr.2013.04.003","article-title":"The BAFF\/APRIL system: emerging functions beyond B cell biology and autoimmunity","volume":"24","author":"Vincent","year":"2013","journal-title":"Cytokine Growth Factor Rev"},{"key":"2025120907362236100_uxaf065-B2","doi-asserted-by":"crossref","first-page":"7333","DOI":"10.1038\/ncomms8333","article-title":"\u03b3-Secretase directly sheds the survival receptor BCMA from plasma cells","volume":"6","author":"Laurent","year":"2015","journal-title":"Nat Commun"},{"key":"2025120907362236100_uxaf065-B3","doi-asserted-by":"crossref","first-page":"e20220214","DOI":"10.1084\/jem.20220214","article-title":"Developing high-affinity decoy receptors to treat multiple myeloma and diffuse large B cell lymphoma","volume":"219","author":"Miao","year":"2022","journal-title":"J Exp Med"},{"key":"2025120907362236100_uxaf065-B4","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1038\/s41375-020-0734-z","article-title":"B-cell maturation antigen (BCMA) in multiple myeloma: rationale for targeting and current therapeutic approaches","volume":"34","author":"Shah","year":"2020","journal-title":"Leukemia"},{"key":"2025120907362236100_uxaf065-B5","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.jaip.2019.08.012","article-title":"Serum B-cell maturation antigen (BCMA) levels differentiate primary antibody deficiencies","volume":"8","author":"Maglione","year":"2020","journal-title":"J Allergy Clin Immunol Pract"},{"key":"2025120907362236100_uxaf065-B6","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1007\/s10875-024-01746-1","article-title":"An exploratory approach of clinically useful biomarkers of cvid by logistic regression","volume":"44","author":"Guerra-Gal\u00e1n","year":"2024","journal-title":"J Clin Immunol"},{"key":"2025120907362236100_uxaf065-B7","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1007\/s10875-022-01231-7","article-title":"SARS-CoV-2 vaccine responses in individuals with antibody deficiency: findings from the COV-AD study","volume":"42","author":"Shields","year":"2022","journal-title":"J Clin Immunol"},{"key":"2025120907362236100_uxaf065-B8","doi-asserted-by":"crossref","first-page":"12211","DOI":"10.1038\/s41598-021-91791-2","article-title":"Variable immunodeficiency score upfront analytical link (VISUAL), a proposal for combined prognostic score at diagnosis of common variable immunodeficiency","volume":"11","author":"Guevara-Hoyer","year":"2021","journal-title":"Sci Rep"},{"key":"2025120907362236100_uxaf065-B9","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1007\/s10875-024-01829-z","article-title":"Outcomes of X-linked agammaglobulinaemia patients","volume":"45","author":"Shillitoe","year":"2024","journal-title":"J Clin Immunol"},{"key":"2025120907362236100_uxaf065-B10","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1007\/s10875-017-0381-y","article-title":"Low IgA and IgM is associated with a higher prevalence of bronchiectasis in primary antibody deficiency","volume":"37","author":"Hodkinson","year":"2017","journal-title":"J Clin Immunol"}],"container-title":["Clinical and Experimental Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/cei\/advance-article-pdf\/doi\/10.1093\/cei\/uxaf065\/64423972\/uxaf065.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/219\/1\/uxaf065\/64423972\/uxaf065.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/219\/1\/uxaf065\/64423972\/uxaf065.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,9]],"date-time":"2025-12-09T12:36:29Z","timestamp":1765283789000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/cei\/article\/doi\/10.1093\/cei\/uxaf065\/8267634"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025]]},"references-count":10,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1,21]]}},"URL":"https:\/\/doi.org\/10.1093\/cei\/uxaf065","relation":{},"ISSN":["0009-9104","1365-2249"],"issn-type":[{"type":"print","value":"0009-9104"},{"type":"electronic","value":"1365-2249"}],"subject":[],"published-other":{"date-parts":[[2025]]},"published":{"date-parts":[[2025]]},"article-number":"uxaf065"}}