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Published on: December 15, 2011
Pediatric Celiac Disease and Selective IgA Deficiency: Unexpected Sequence of Events
Maria Christine Ernst Andersen1, Stine Dydensborg Sander1, Gunvor Madsen2
1Hans Christian Andersen Children's Hospital, Odense University Hospital, Kloevervaenget 23C, 5000, Odense C, Denmark.
Purpose:
Selective IgA deficiency (IgAD) is the most common primary immunodeficiency, frequently leading to only minor clinical complaints. IgAD may be associated with autoimmune diseases such as celiac disease (CeD). Although IgAD is thought to precede CeD and autoimmunity, the association between the two conditions has not been clarified.
Methods:
Routine techniques were used to measure serum IgA and celiac diagnostic markers as transglutaminase 2 IgA (TG2-IgA) and deamidated gliadin IgG and for immunohistochemistry for IgG, IgM, and IgA.
Results:
We report two childhood cases of complete IgA deficiency that evolved after the diagnosis of CeD and the start of a gluten-free diet. Histology showed persistence of IgA in the intestinal mucosa.
Conclusion:
Both children with CeD showed IgA deficiency that unexpectedly developed after the initiation of a gluten-free diet. This supports IgA deficiency as a process that develops gradually and occurs due to specific defects in immunoregulation.
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