PURPOSE: Limited information exists on the natural history, comorbid conditions and immunologic features of Selective IgM immunodeficiency (SIgMID) in children. We reviewed and compared previously published pediatric cases in addition to reporting 2 new cases.
METHODS: Published literature selected from the English only PubMed database from 1966 and references from bibliographies of relevant articles were utilized. Previously reported cases were reviewed and included in a database with 2 new cases obtained from a retrospective analysis from an allergy and immunology practice.
RESULTS: Previously reported 49 cases of SIgMID presented with recurrent 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. Mean age of presentation was 6.0 ± 4.7 years. No malignancies were reported and 3.9% had autoimmune disease. Two patients (ages 10 and 12) were identified with SIgMID among a database of 6,300 active pediatric patients (0.03%). The serum IgM levels were 29 and 30 ml/dL (both <2 SD below age adjusted means). Presenting symptoms were: asthma, vasomotor rhinitis, and recurrent respiratory infections. In the now 51 cases reported, none have developed lymphoproliferative disease nor evolved into panhypogammaglobulinemia, although 4 fatalities have been reported, all in children ≤; 8 years of age.
CONCLUSION: 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.
CLINICAL IMPLICATIONS: SIgMID should be considered in the differential diagnosis of a child with recurrent infections.
DISCLOSURE: Marc Goldstein, No Financial Disclosure Information; No Product/Research Disclosure Information