Immunoglobulin A deficiency in celiac disease.

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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 .