Pediatric Celiac Disease and Selective IgA Deficiency: Unexpected Sequence of Events

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 t...

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Published inJournal of clinical immunology Vol. 42; no. 6; pp. 1342 - 1346
Main Authors Andersen, Maria Christine Ernst, Sander, Stine Dydensborg, Madsen, Gunvor, Lillevang, Søren T., Murray, Joseph, Husby, Steffen
Format Journal Article
LanguageEnglish
Published New York Springer US 01.08.2022
Springer Nature B.V
Subjects
Antibodies
Autoimmune diseases
Autoimmunity
Biomarkers
Biomedical and Life Sciences
Biomedicine
Biopsy
Birth weight
Celiac disease
Children
Constipation
Diet
Endoscopy
Gastroenterology
Gliadin
Gluten
Hypotheses
Immune system
Immunodeficiency
Immunoglobulin A
Immunoglobulin G
Immunoglobulin M
Immunohistochemistry
Immunology
Immunoregulation
Infectious Diseases
Internal Medicine
Medical Microbiology
Nutrient deficiency
Original Article
Patients
Pediatrics
Primary immunodeficiencies
Serology
Small intestine
Transglutaminase 2
Celiac disease
IgA deficiency
Children
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Summary: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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ISSN:0271-9142
1573-2592
DOI:10.1007/s10875-022-01297-3