ReviewClinical reviews in allergy & immunology2014
Primary selective IgM deficiency: an ignored immunodeficiency.
Review in Clinical reviews in allergy & immunology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 87 citations in OpenAlex.
- IRAK-4 and MyD88 deficiencies impair IgM responses against T-independent bacterial antigens.Blood · 2014Trial
- Low IgM Levels in Adult IEI: Classification Challenges and Clinical Implications.Journal of clinical immunology · 2026Article
- Pediatric selective IgM deficiency: clinical features and a preliminary risk index for immunoglobulin replacement therapy.Immunologic research · 2025Article
- Expanding the Spectrum of Selective IgM Deficiency: From Infections to Immune Dysregulation.International journal of molecular sciences · 2025Article
- Understanding IgM Structure and Biology to Engineer New Antibody Therapeutics.BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy · 2025Review
- Selective IgM Deficiency: Evidence, Controversies, and Gaps.Diagnostics (Basel, Switzerland) · 2023Review
- Deficient Complement Opsonization Impairs Mycobacterium avium Killing by Neutrophils in Cystic Fibrosis.Microbiology spectrum · 2023Article
- B cells defined by immunoglobulin isotypes.Clinical and experimental immunology · 2022Article
- Collagenous Gastritis in Primary Selective IgM Deficiency: Transition to EBV+ Gastric Adenocarcinoma.Case reports in immunology · 2021Article
- Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency.Case reports in immunology · 2021Article
- Update on Infections in Primary Antibody Deficiencies.Frontiers in immunology · 2021Review
- Structure, Function, and Therapeutic Use of IgM Antibodies.Antibodies (Basel, Switzerland) · 2020Review
- Neonatal Exposure to Commensal-Bacteria-Derived Antigens Directs Polysaccharide-Specific B-1 B Cell Repertoire Development.Immunity · 2020Article
- The epidemiology and clinical features of selective immunoglobulin M deficiency: A single-center study in China.Journal of clinical laboratory analysis · 2020Article
- Glycan Reactive Natural Antibodies and Viral Immunity.Viral immunology · 2020Review
- Methylmercury and long chain polyunsaturated fatty acids are associated with immune dysregulation in young adults from the Seychelles child development study.Environmental research · 2020Article
- IgM Plasma Cells Reside in Healthy Skin and Accumulate with Chronic Inflammation.The Journal of investigative dermatology · 2019Article
- Acute-onset Autoimmune Hepatitis in a Patient with Selective Immunoglobulin M Deficiency.Internal medicine (Tokyo, Japan) · 2019Article
- Challenges in investigating patients with isolated decreased serum IgM: The SIMcal study.Scandinavian journal of immunology · 2019Article
- Defining Primary Selective IgM Deficiency.Journal of clinical immunology · 2019Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Immunoglobulin M (IgM) provides the initial response to foreign antigen and plays a regulatory role in subsequent immune response development, accelerating the production of high-affinity IgG. Though selective IgM deficiency was described more than 45 years ago in children with fulminant meningococcal septicemia, it has been largely an ignored primary immunodeficiency. It appears to be more common than originally realized. Selective IgM deficiency is observed in both children and adults with no gender bias. The most common clinical manifestation of selective IgM deficiency is infections with extracellular and intracellular bacteria, viruses, and fungi. Allergic diatheses are the second commonest presentation of selective IgM deficiency. There is an increased prevalence of autoimmune diseases, which in both humans and mice appear to be secondary to selective IgM deficiency rather IgM deficiency secondary to autoimmune diseases. Selective IgM deficiency, in some cases, is associated with 22q11.2 chromosome deletion and few familial cases of selective IgM deficiency have been reported. Innate immunity is relatively intact. T cells, T cell subsets, and T cell functions are normal. However, several patients with selective IgM deficiency and T cell and NK cell defects with Mycobacterium avium intracellulare infections have been reported. In a subset of patients with selective IgM deficiency circulating IgM+ B cells are decreased or completely lacking. Specific IgG antibody responses against pneumococcus polysaccharides are impaired in a subset of patients with selective IgM deficiency. The pathogenesis of selective IgM deficiency is unclear; decreased T helper activity, increased isotype-specific suppressor T cell activity, and intrinsic B cell defects have been reported. Patients with selective IgM deficiency and impaired pneumococcal antibody responses appear to respond to immunoglobulin therapy.
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