PMID- 28928736
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240326
IS  - 1664-3224 (Print)
IS  - 1664-3224 (Electronic)
IS  - 1664-3224 (Linking)
VI  - 8
DP  - 2017
TI  - Selective IgM Deficiency-An Underestimated Primary Immunodeficiency.
PG  - 1056
LID - 10.3389/fimmu.2017.01056 [doi]
LID - 1056
AB  - 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.
FAU - Gupta, Sudhir
AU  - Gupta S
AD  - Program in Primary Immunodeficiency and Aging, Division of Basic and Clinical 
      Immunology, University of California at Irvine, Irvine, CA, United States.
FAU - Gupta, Ankmalika
AU  - Gupta A
AD  - Program in Primary Immunodeficiency and Aging, Division of Basic and Clinical 
      Immunology, University of California at Irvine, Irvine, CA, United States.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20170905
PL  - Switzerland
TA  - Front Immunol
JT  - Frontiers in immunology
JID - 101560960
PMC - PMC5591887
OTO - NOTNLM
OT  - Breg
OT  - CD4 Treg
OT  - CD8 Treg
OT  - autoimmunity
OT  - specific antibody deficiency
EDAT- 2017/09/21 06:00
MHDA- 2017/09/21 06:01
PMCR- 2017/01/01
CRDT- 2017/09/21 06:00
PHST- 2017/06/28 00:00 [received]
PHST- 2017/08/14 00:00 [accepted]
PHST- 2017/09/21 06:00 [entrez]
PHST- 2017/09/21 06:00 [pubmed]
PHST- 2017/09/21 06:01 [medline]
PHST- 2017/01/01 00:00 [pmc-release]
AID - 10.3389/fimmu.2017.01056 [doi]
PST - epublish
SO  - Front Immunol. 2017 Sep 5;8:1056. doi: 10.3389/fimmu.2017.01056. eCollection 
      2017.