Selective IgM deficiency associated with adult-onset Still disease

Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency

“…We searched the English Literature and we found sex cases of patients diagnosed with primary sIgMD and autoimmune diseases. Two of them had SLE2 3 with single cases of Hashimoto’ s disease,4 glomerulonephritis,5 autoimmune hepatitis6 and adult-onset Still Disease 7. The mean age at diagnosis was 55 years.…”

“…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.…”

Selective IgM Deficiency: Evidence, Controversies, and Gaps

“…Goldstein et al reported autoimmune diseases in 14% of patients with SIgMD [19], although, in children, they are less frequent [46]. Autoimmune diseases, including systemic lupus erythematosus [60], Hashimoto thyroiditis [61], autoimmune thrombocytopenia [62], autoimmune glomerulonephritis [62], autoimmune hepatitis [63], juvenile idiopathic arthritis [64], and rheumatoid arthritis have been described as comorbidities in SIgMD. Also, chronic recurrent multifocal osteomyelitis [65], rheumatic heart disease, psoriasis, and scleroderma [15] have been reported.…”