eprintid: 10065788 rev_number: 19 eprint_status: archive userid: 608 dir: disk0/10/06/57/88 datestamp: 2019-01-16 19:24:31 lastmod: 2021-09-26 22:34:25 status_changed: 2019-01-16 19:24:31 type: article metadata_visibility: show creators_name: Campochiaro, C creators_name: Atay, S creators_name: Clark, KEN creators_name: Ong, V creators_name: Denton, CP title: Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: G90 keywords: connective tissue disease, immunology note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. abstract: Selective immunoglobulin M deficiency (sIgMD) is an immunodeficiency with undefined pathogenesis and commonly presenting with recurrent infections. 1 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. 2-7 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. date: 2019-01-03 date_type: published official_url: http://dx.doi.org/10.1136/bcr-2017-223180 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green article_type_text: Journal Article verified: verified_manual elements_id: 1620125 doi: 10.1136/bcr-2017-223180 pii: 12/1/e223180 lyricists_name: Clark, Kristina lyricists_name: Denton, Christopher lyricists_name: Ong, Voon lyricists_id: KENCL47 lyricists_id: CPDEN87 lyricists_id: VONGX45 actors_name: Denton, Christopher actors_id: CPDEN87 actors_role: owner full_text_status: public publication: BMJ Case Reports volume: 12 number: 1 article_number: e223180 event_location: England issn: 1757-790X citation: Campochiaro, C; Atay, S; Clark, KEN; Ong, V; Denton, CP; (2019) Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency. BMJ Case Reports , 12 (1) , Article e223180. 10.1136/bcr-2017-223180 <https://doi.org/10.1136/bcr-2017-223180>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10065788/1/Accepted%20version%20BMJ%20CR%202018.pdf