@article{discovery10065788, note = {This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.}, month = {January}, volume = {12}, number = {1}, journal = {BMJ Case Reports}, year = {2019}, title = {Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency}, keywords = {connective tissue disease, immunology}, url = {http://dx.doi.org/10.1136/bcr-2017-223180}, author = {Campochiaro, C and Atay, S and Clark, KEN and Ong, V and Denton, CP}, issn = {1757-790X}, 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.} }