{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T01:21:24Z","timestamp":1768612884601,"version":"3.49.0"},"reference-count":45,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2017,10,27]],"date-time":"2017-10-27T00:00:00Z","timestamp":1509062400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,1,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Isolated decreased serum-immunoglobulin (Ig)M has been associated with severe and\/or recurrent infections, atopy and autoimmunity. However, the reported high prevalence of clinical problems in IgM-deficient patients may reflect the skewed tertiary centre population studied so far. Also, many papers on IgM deficiency have included patients with more abnormalities than simply IgM-deficiency. We studied truly selective primary IgM deficiency according to the diagnostic criteria of the European Society for Immunodeficiencies (ESID) (true sIgMdef) by reviewing the literature (261 patients with primary decreased serum-IgM in 46 papers) and analysing retrospectively all patients with decreased serum-IgM in a large teaching hospital in 's-Hertogenbosch, the Netherlands [1 July 2005\u201323 March 2016; n = 8049 IgM &amp;lt;\u00a00\u00b74 g\/l; n = 2064 solitary (IgG+IgA normal\/IgM\u00a0&amp;lt;\u00a0age-matched reference)]. A total of 359 of 2064 (17%) cases from our cohort had primary isolated decreased serum-IgM, proven persistent in 45 of 359 (13%) cases; their medical charts were reviewed. Our main finding is that true sIgMdef is probably very rare. Only six of 261 (2%) literature cases and three of 45 (7%) cases from our cohort fulfilled the ESID criteria completely; 63 of 261 (24%) literature cases also had other immunological abnormalities and fulfilled the criteria for unclassified antibody deficiencies (unPAD) instead. The diagnosis was often uncertain (possible sIgMdef): data on IgG subclasses and\/or vaccination responses were lacking in 192 of 261 (74%) literature cases and 42 of 45 (93%) cases from our cohort. Our results also illustrate the clinical challenge of determining the relevance of a serum sample with decreased IgM; a larger cohort of true sIgMdef patients is needed to explore fully its clinical consequences. The ESID online Registry would be a useful tool for this.<\/jats:p>","DOI":"10.1111\/cei.13065","type":"journal-article","created":{"date-parts":[[2017,10,6]],"date-time":"2017-10-06T12:42:34Z","timestamp":1507293754000},"page":"203-211","source":"Crossref","is-referenced-by-count":19,"title":["Truly selective primary IgM deficiency is probably very rare"],"prefix":"10.1093","volume":"191","author":[{"given":"L M A","family":"Janssen","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T","family":"Macken","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M C W","family":"Creemers","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J F M","family":"Pruijt","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J J J","family":"Eijk","sequence":"additional","affiliation":[{"name":"Department of Neurology, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"de Vries","sequence":"additional","affiliation":[{"name":"Department of Tranzo, Tilburg University, Tilburg, the Netherlands"},{"name":"Laboratory for Medical Microbiology and Immunology, Elisabeth Tweesteden Hospital, Tilburg, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2017,10,27]]},"reference":[{"key":"2021120808203648900_cei13065-bib-0001","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1007\/s12016-013-8375-x","article-title":"Primary selective IgM deficiency: an ignored immunodeficiency","volume":"46","author":"Louis","year":"2014","journal-title":"Clin Rev Allergy Immunol"},{"key":"2021120808203648900_cei13065-bib-0002","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.humimm.2015.09.051","article-title":"The prevalence of Selective Immunoglobulin M Deficiency (SIgMD) in Iranian volunteer blood donors","volume":"77","author":"Entezari","year":"2016","journal-title":"Hum Immunol"},{"key":"2021120808203648900_cei13065-bib-0003","doi-asserted-by":"crossref","first-page":"e47990","DOI":"10.1371\/journal.pone.0047990","article-title":"Genome-wide scan identifies variant in TNFSF13 associated with serum IgM in a healthy Chinese male population","volume":"7","author":"Yang","year":"2012","journal-title":"PLOS ONE"},{"key":"2021120808203648900_cei13065-bib-0004","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1159\/000222682","article-title":"Clinical and immunological features in IgM deficiency","volume":"150","author":"Yel","year":"2009","journal-title":"Int Arch Allergy Immunol"},{"key":"2021120808203648900_cei13065-bib-0005","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1016\/S1081-1206(10)60962-3","article-title":"Selective IgM immunodeficiency: retrospective analysis of 36 adult patients with review of the literature","volume":"97","author":"Goldstein","year":"2006","journal-title":"Ann Allergy Asthma Immunol"},{"key":"2021120808203648900_cei13065-bib-0006","doi-asserted-by":"crossref","first-page":"624850","DOI":"10.1155\/2008\/624850","article-title":"Pediatric selective IgM immunodeficiency","volume":"2008","author":"Goldstein","year":"2008","journal-title":"Clin Dev Immunol"},{"key":"2021120808203648900_cei13065-bib-0007","first-page":"547","article-title":"IgM deficiency: clinical spectrum and immunologic assessment","volume":"62","author":"Guill","year":"1989","journal-title":"Ann Allergy"},{"key":"2021120808203648900_cei13065-bib-0008","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/j.ijmyco.2015.11.002","article-title":"Selective immunoglobulin M deficiency in an adult with miliary tuberculosis: a clinically interesting coexistence. 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