Pediatric Selective IgM Immunodeficiency

Abstract

Objective. Limited information exists on features of pediatric Selective IgM immunodeficiency (SIgMID). Previously published pediatric cases and 2 new cases are reviewed. Methods. English literature from PubMed and references from relevant articles were reviewed. Previously reported cases and 2 new cases from an allergy/immunology practice were analyzed. Results. Forty-nine reported cases of SIgMID presented with respiratory infections (77.6%), gastrointestinal disease (16.3%), skin disease (12.2%), and meningitis (8.2%). Mean serum IgM level was 16.5 ± 13.8 mg/dL. Two patients were identified with SIgMID among 6300 active pediatric patients (0.03%) presenting with asthma, vasomotor rhinitis, and recurrent respiratory infections. In the 51 cases reported, none developed lymphoproliferative disease nor evolved into panhypogammaglobulinemia; four fatalities were reported. Conclusions. The prevalence of SIgMID in our pediatric population was 0.03%. In general, respiratory infections are the common comorbid conditions. Death and autoimmune disease are uncommon complications of pediatric SIgMID.

Altered immunoglobulin profiles in children with Tourette syndrome

“…Both IgG3 and monomeric IgM also play a role in phagocytosis (Meyts et al, 2006; Shibuya et al, 2000). Since IgG3 and IgM are involved in immune responses against multiple microorganisms (Goldstein et al, 2008; Noyes et al, 1986; Oxelius et al, 1986), decreased levels may compromise the immune defense to diverse pathogens.…”

T-Independent Antibody Responses to T-Dependent Antigens: A Novel Follicular Dendritic Cell-Dependent Activity

“…Moreover, its multimeric structure makes it a strong complement activator; a single bound IgM pentamer can trigger the classical pathway of complement activation and can lyse a RBC, whereas ϳ1000 IgG molecules are required to accomplish the same (58). In synergy with complement, IgM offers immediate protection to systemic bacterial and viral infections (59), and patients with normal IgG levels but selective IgM deficiency suffer from morbid recurrent upper respiratory tract infections, asthma, celiac disease, and meningitis (60,61). Other potential applications include countering the negative effect of regulatory T cells and the nonresponder state as a consequence of a limited MHC II repertoire that may be unable to load certain peptides.…”

sIgM–FcμR Interactions Regulate Early B Cell Activation and Plasma Cell Development after Influenza Virus Infection

“…Selective IgM-deficiency has been associated with increased morbidity and mortality from various bacterial, viral, fungal and parasitic infections in humans (11–13). Consistent with these findings, sIgM deficient (μs −/− ) mice had significantly increased viral loads and decreased survival rates compared to wild-type controls after influenza virus infection (14, 15), as well as in response to other pathogens (5, 16–25).…”