Although secretory immunoglobulin A (IgA) is important in mucosal immunity,
selective IgA deficiency is the most common primary immunodeficiency of hu
mans. In most cases this defect is not associated with any illness. The rea
sons for this are unknown, but other immunological compensations might prov
ide sufficient or complete restitution. Alternatively, it is possible that
IgA deficiency alone may not predispose to disease, but additional immunolo
gical abnormalities might be present in symptomatic individuals. Some IgA-d
eficient individuals have a reduced antibody response to immunizations (eve
n with normal IgG and IgM levels) and others have deficient responses to ba
cterial polysaccharides when IgG subclass levels are normal. The physiologi
cal role of IgA. the frequency and causes of IgA deficiency, the diseases a
ssociated with its absence, and current limited understanding of the pathog
enesis of selective IgA deficiency will be reviewed.