TY - JOUR TI - Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency AV - public VL - 12 Y1 - 2019/01/03/ N1 - This version is the author accepted manuscript. For information on re-use, please refer to the publisher?s terms and conditions. IS - 1 ID - discovery10065788 N2 - 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. SN - 1757-790X UR - http://dx.doi.org/10.1136/bcr-2017-223180 JF - BMJ Case Reports KW - connective tissue disease KW - immunology A1 - Campochiaro, C A1 - Atay, S A1 - Clark, KEN A1 - Ong, V A1 - Denton, CP ER -