Natural History of Selective Antibody Deficiency to Bacterial Polysaccharide Antigens in Children

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Abstract
Children diagnosed with selective antibody deficiency to bacterial polysaccharide antigens over the last 10 years were analyzed in a retrospective study to examine the clinical characteristics and natural history for children with this disease. A total of 120 children aged more than 2 years were diagnosed based on a history of recurrent infections and failure to respond to a majority of serotypes following Streptococcus pneumoniae immunizations. An infectious history included recurrent otitis media (91%), sinusitis (97%), pneumonia (57%), and invasive infection (5%). Immune evaluation revealed 11% with IgA deficiency, 19% with IgG-2 deficiency, and average response to only 35% of S. pneumoniae serotypes. A total of 34 of 120 patients received intravenous immunoglobulin (IVIG) because of recurrent infections despite prophylactic antibiotics. The IVIG group had a greater percentage of abnormal responses to immunization, 73% versus 61% of serotypes tested (p < 0.01). Repeat immune studies after a mean interval of 3.1 years showed 50% of patients developed normal responses. Children with selective antibody deficiency to polysaccharide antigens present with recurrent upper and lower respiratory tract infections, but rarely with invasive disease. Natural history indicates that most patients will normalize their antibody responses to bacterial polysaccharides by age 5 to 6 years, whereas in a subgroup the defect may be permanent.