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2017 ; 8
(ä): 1056
Nephropedia Template TP
Front Immunol
2017[]; 8
(ä): 1056
PMID28928736
show ga
Although selective IgM deficiency (SIGMD) was described almost five decades ago,
it was largely ignored as a primary immunodeficiency. SIGMD is defined as serum
IgM levels below two SD of mean with normal serum IgG and IgA. It appears to be
more common than originally realized. SIGMD is observed in both children and
adults. Patients with SIGMD may be asymptomatic; however, approximately 80% of
patients with SIGMD present with infections with bacteria, viruses, fungi, and
protozoa. There is an increased frequency of allergic and autoimmune diseases in
SIGMD. A number of B cell subset abnormalities have been reported and impaired
specific antibodies to Streptococcus pneumoniae responses are observed in more
than 45% of cases. Innate immunity, T cells, T cell subsets, and T cell functions
are essentially normal. The pathogenesis of SIGMD remains unclear. Mice
selectively deficient in secreted IgM are also unable to control infections from
bacterial, viral, and fungal pathogens, and develop autoimmunity. Immunological
and clinical similarities and differences between mouse models of deficiency of
secreted IgM and humans with SIGMD have been discussed. Patients with SIGMD
presenting with recurrent infections and specific antibody deficiency responses
appear to improve clinically on immunoglobulin therapy.