Campochiaro Corrado, Atay Sirin, Clark Kristina E N, Ong Voon, Denton Christopher P
Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK.
Department of Experimental Rheumatology, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK.
BMJ Case Rep. 2019 Jan 3;12(1):e223180. doi: 10.1136/bcr-2017-223180.
Selective immunoglobulin M deficiency (sIgMD) is an immunodeficiency with undefined pathogenesis and commonly presenting with recurrent infections. 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. 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.
选择性免疫球蛋白M缺乏症（sIgMD）是一种发病机制不明的免疫缺陷病，常表现为反复感染。欧洲免疫缺陷学会登记处将sIgMD定义为血清IgM水平反复低于正常水平的2个标准差，同时血清IgA、IgG和IgG亚类水平正常，疫苗接种反应正常，无T细胞缺陷且无致病外部因素。它很少也可与自身免疫性疾病相关。在此，我们描述了一名原发性sIgMD患者，该患者患有多种自身免疫性疾病但无反复感染史，并且我们提供了一篇关于sIgMD和自身免疫性疾病的简短文献综述。
