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Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency
  1. Corrado Campochiaro1,
  2. Sirin Atay1,
  3. Kristina E N Clark1,
  4. Voon Ong2 and
  5. Christopher P Denton2
  1. 1 Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK
  2. 2 Department of Experimental Rheumatology, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK
  1. Correspondence to Dr Christopher P Denton, c.denton{at}


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.

  • connective tissue disease
  • immunology
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  • Contributors CC and SA: wrote the manuscript. VO, CPD and KENC revised it and substantially contributed to the latest version.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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