title: Immunoglobulin A deficiency in celiac disease.
abstract: OBJECTIVE
  To determine the prevalence and significance of immunoglobulin A (IgA) deficiency and partial deficiency in patients with celiac disease (CD).
  
  
  BACKGROUND
  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.
  
  
  METHODS
  A retrospective, cohort study of 1498 adults and 317 children seen in a University Medical Center was conducted.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Medicine, Columbia University Medical Center, NY 10032, USA.
authors.forename: Marisa A
authors.initials: MA
authors.lastname: Chow
authors.forename: Benjamin
authors.initials: B
authors.lastname: Lebwohl
authors.forename: Norelle Rizkalla
authors.initials: NR
authors.lastname: Reilly
authors.forename: Peter H R
authors.initials: PH
authors.lastname: Green
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000453:epidemiology; D002446:Celiac Disease / Q000453:epidemiology* / Q000276:immunology* / Q000473:pathology; D002648:Child; D003967:Diarrhea / Q000209:etiology; D055050:Diet, Gluten-Free; D005260:Female; D019204:GTP-Binding Proteins; D005903:Gliadin / Q000276:immunology; D006801:Humans; D017098:IgA Deficiency / Q000453:epidemiology* / Q000276:immunology; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D015995:Prevalence; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D012189:Retrospective Studies; D011503:Transglutaminases / Q000276:immunology
chemical_list: D007074:Immunoglobulin G; D005903:Gliadin; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D011503:Transglutaminases; D019204:GTP-Binding Proteins