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

OBJECTIVE

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.

DATA SOURCES

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.

STUDY SELECTION

Reported adult SIgMID cases were reviewed and included in a comparative database against our cohort.

RESULTS

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.

CONCLUSIONS

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.