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To determine the prevalence and significance of immunoglobulin A (IgA) deficiency and partial deficiency in patients with celiac disease (CD).Selective IgA deficiency is a common primary immunoglobulin deficiency and has a higher prevalence in patients with CD. The prevalence and significance of IgA deficiency and partial deficiency in patients with CD in the United States has not previously been examined.A retrospective, cohort study of 1498 adults and 317 children seen in a University Medical Center was conducted.There were 26 patients (22 adults, 4 children) with CD who were IgA deficient and 11 (9 adults, 2 children) with CD who were partially IgA deficient. The prevalence of IgA deficiency/partial deficiency was similar among adults and children (2.1% and 1.9%, respectively, P=0.99). Among adults, concomitant autoimmune disease was present in 29% of IgA-deficient/partially deficient patients versus 12% of CD patients with normal IgA levels (P=0.0081). All 4 IgA-deficient patients who had persistently positive IgG celiac serologies while adherent to a gluten-free diet and were rebiopsied had a normal repeat biopsy. Both positive tissue transglutaminase IgG and antigliadin IgG were found in these patients.Selective IgA deficiency/partial deficiency is present in 2% of CD patients at this referral center and is equally prevalent among adults and children. IgA-deficient/partially deficient adults had a higher prevalence of concomitant autoimmune disease than those without IgA deficiency. In patients who are IgA deficient, IgG serologies may be persistently elevated despite histologic recovery.