{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:33:27Z","timestamp":1787016807357,"version":"build-2736575974"},"reference-count":22,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2018,12,17]]},"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:p>\n                    Selective immunoglobulin M deficiency (sIgMD) is an immunodeficiency with undefined pathogenesis and commonly presenting with recurrent infections.\n                    <jats:sup>1<\/jats:sup>\n                    The European Society for Immunodeficiencies Registry defines sIgMD as a serum IgM level repeatedly below 2 SD of normal with normal levels of serum IgA, IgG and IgG subclasses, normal vaccination responses, absence of T-cell defects and absence of causative external factors. Rarely it can also be associated with autoimmune diseases.\n                    <jats:sup>2\u20137<\/jats:sup>\n                    Here we describe a patient with primary sIgMD; who presented with multiple autoimmune diseases without a history of recurrent infections and we provide a short literature review on sIgMD and autoimmune diseases.\n                  <\/jats:p>","DOI":"10.1136\/bcr-2017-223180","type":"journal-article","created":{"date-parts":[[2019,1,4]],"date-time":"2019-01-04T05:15:58Z","timestamp":1546578958000},"page":"e223180","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":12,"title":["Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency"],"prefix":"10.1136","volume":"12","author":[{"given":"Corrado","family":"Campochiaro","sequence":"first","affiliation":[{"name":"Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sirin","family":"Atay","sequence":"additional","affiliation":[{"name":"Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina E N","family":"Clark","sequence":"additional","affiliation":[{"name":"Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Voon","family":"Ong","sequence":"additional","affiliation":[{"name":"Department of Experimental Rheumatology, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher P","family":"Denton","sequence":"additional","affiliation":[{"name":"Department of Experimental Rheumatology, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2019,1,3]]},"reference":[{"key":"2025082722503301000_12.1.e223180.1","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1007\/s10875-017-0420-8","article-title":"Selective IgM deficiency: clinical and laboratory features of 17 patients and a review of the literature","volume":"37","author":"Chovancova","year":"2017","journal-title":"J Clin Immunol"},{"key":"2025082722503301000_12.1.e223180.2","doi-asserted-by":"crossref","first-page":"185","DOI":"10.2329\/perio.38.Supplement1_185","article-title":"[A case of selective IgM deficiency associated with systemic lupus erythematosus]","volume":"38","author":"Tanaka","year":"1996","journal-title":"Nihon Jinzo Gakkai Shi"},{"key":"2025082722503301000_12.1.e223180.3","doi-asserted-by":"publisher","DOI":"10.3109\/08916930109001959"},{"key":"2025082722503301000_12.1.e223180.4","doi-asserted-by":"crossref","first-page":"302","DOI":"10.2169\/internalmedicine.32.302","article-title":"Selective IgM deficiency in a patient with Hashimoto\u2019s disease","volume":"32","author":"Kimura","year":"1993","journal-title":"Intern Med"},{"key":"2025082722503301000_12.1.e223180.5","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1007\/s10157-008-0049-2","article-title":"A case of selective immunoglobulin M deficiency and autoimmune glomerulonephritis","volume":"12","author":"Antar","year":"2008","journal-title":"Clin Exp Nephrol"},{"key":"2025082722503301000_12.1.e223180.6","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1159\/000343583","article-title":"A novel type of selective immunoglobulin m deficiency in a patient with autoimmune liver cirrhosis with recurrent hepatocellular carcinoma: a case report and review of the literature","volume":"161","author":"Arahata","year":"2013","journal-title":"Int Arch Allergy Immunol"},{"key":"2025082722503301000_12.1.e223180.7","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1016\/j.anai.2018.01.001","article-title":"Selective IgM deficiency associated with adult-onset Still disease","volume":"120","author":"Oh","year":"2018","journal-title":"Ann Allergy Asthma Immunol"},{"key":"2025082722503301000_12.1.e223180.8","unstructured":"Krishnaswamy G . Selective IgM deficiency\u00a0UpToDate. 2017."},{"key":"2025082722503301000_12.1.e223180.9","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-013-8375-x"},{"key":"2025082722503301000_12.1.e223180.10","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. A case report and review of the literature","volume":"5","author":"Hassanein","year":"2016","journal-title":"Int J Mycobacteriol"},{"key":"2025082722503301000_12.1.e223180.11","first-page":"15","article-title":"Syndrome of selective IgM deficiency with severe T cell deficiency associated with disseminated cutaneous mycobacterium avium intracellulaire infection","volume":"4","author":"Gharib","year":"2015","journal-title":"Am J Clin Exp Immunol"},{"key":"2025082722503301000_12.1.e223180.12","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.jmii.2013.01.009","article-title":"Selective IgM deficiency in an adult presenting with\u00a0Streptococcus pneumoniae septic arthritis","volume":"49","author":"Phuphuakrat","year":"2016","journal-title":"J Microbiol Immunol Infect"},{"key":"2025082722503301000_12.1.e223180.13","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1159\/000199725","article-title":"Selective immunoglobulin M deficiency presenting with recurrent impetigo: a case report and review of the literature","volume":"149","author":"Belgemen","year":"2009","journal-title":"Int Arch Allergy Immunol"},{"key":"2025082722503301000_12.1.e223180.14","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1007\/s00296-008-0788-0","article-title":"Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency","volume":"29","author":"Makay","year":"2009","journal-title":"Rheumatol Int"},{"key":"2025082722503301000_12.1.e223180.15","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1067\/mpa.2001.111018","article-title":"Multiple recurrent hordeola associated with selective IgM deficiency","volume":"5","author":"Kiratli","year":"2001","journal-title":"J Aapos"},{"key":"2025082722503301000_12.1.e223180.16","doi-asserted-by":"publisher","DOI":"10.1016\/S0091-6749(75)80006-6"},{"key":"2025082722503301000_12.1.e223180.17","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)60962-3"},{"key":"2025082722503301000_12.1.e223180.18","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":"2025082722503301000_12.1.e223180.19","unstructured":"Kahn MF , Appelboom T , Sharp S . et al In: Kahn MF , Peltier AP , Mayer O , Piette JC , . eds. 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