Selective IgM immunodeficiency: retrospective analysis of 36 adult patients with review of the literature.

To review and compare previously reported cases of selective IgM immunodeficiency (SIgMID) with the largest adult cohort obtained from a retrospective analysis of an allergy and immunology practice. Publications were selected from the English-only PubMed database (1966-2005) using the following keywords: IgM immunodeficiency alone and in combination with celiac disease, autoimmune disease, malignancy, and infection. Bibliographic references of relevant articles were used. Reported adult SIgMID cases were reviewed and included in a comparative database against our cohort. Previously described patients with SIgMID include 155 adults and 157 patients of unspecified age. Thirty-six adult patients were identified with SIgMID from a database of 13,700 active adult patients (0.26%, 1:385). The mean +/- SD serum IgM level was 29.74 +/- 8.68 mg/dL (1 SD). The mean +/- SD age at the time of diagnosis of SIgMID was 55 +/- 13.5 years. Frequency of presenting symptoms included the following: recurrent upper respiratory tract infections, 77%; asthma, 47%; allergic rhinitis, 36%; vasomotor rhinitis, 19%; angioedema, 14%; and anaphylaxis, 11%. Serologically, 13% of patients had positive antinuclear antibodies (ANAs), 5% had serologic evidence of celiac disease, and nearly all had non-AB blood type. Patients also had low levels of IgM isohemagglutinins. No patients developed lymphoproliferative disease or panhypogammaglobulinemia, and none died of life-threatening infections, malignancy, or fulminant autoimmune-mediated diseases during a mean follow-up period of 3.7 years. The prevalence of SIgMID in our adult population was 0.26% and may be more common than previously thought. Non-life-threatening respiratory disorders were common comorbid conditions.