Description of a cohort of patients with selective polysaccharide antibody deficiency

Abstract

Rationale Selective polysaccharide antibody deficiency has been classified as part of the spectrum of common variable immunodeficiency. The clinical characteristics of patients with this immune dysfunction have not been fully reported. Methods Mayo Clinic patient records (who gave consent to be included in research) were reviewed between 1996 and 1999. 40 patients were identified with this diagnosis (defined as <9 of 12 protective-one month-post vaccination pneumococcal serologies). Results Patients in this cohort were referred to and evaluated at the primary immune deficiency clinic. All had an eleven-year history of recurrent infections prior to evaluation. The mean age of females at the time of evaluation was 44 ∗ 20 years (mean ∗ SD) and males 41 ∗ 21. The female to male ratio was 2:1. Diseases at presentation included pneumonia (70%), sinusitis (62.5%), otitis media (45%), thyroiditis (20%), other autoimmune disease (22.5%), bronchiectasis (15%), meningitis (15%), sepsis (12.5%), and lymphadenopathy (7.5%). Autoimmune diagnoses were thyroiditis, ITP, pernicious anemia, hemolytic anemia, neutropenia, celiac disease, neurosarcoidosis and inflammatory arthritis. Mean immunoglobulin isotype and IgG subclass levels were normal. Protective immune responses to pneumococcal serotypes were most common to 7F and 19F and least common to 9V. Family members had a history of immunodeficiency (3), infection (4), thyroid disease (1), and hematological malignancy (4). IGIV was required in a third of the patients. Conclusions Patients with recurrent infections should have a functional evaluation of their immune system. Selective antibody deficiency could be present despite a normal initial immune workup. Autoimmune conditions are associated with this condition.