Pediatric Typical Vs. Atypical Celiac Disease: Correlation of Duodenal Histology With Tissue Transglutaminase Levels

Background: Both the clinical presentation and the degree of mucosal damage in coeliac disease vary greatly. In view of conflicting information as to whether the mode of presentation correlates with the degree of villous atrophy, we reviewed a 103 pediatric patient with coeliac disease. This study also aimed to determine the correlation of anti tTG level and the degree of mucosal damage in typical and atypical cases.  Methods: The medical records of patients with newly diagnosed CD during the period of 2006 - 2010 were retrospectively reviewed.  Results: A total of 103 patients fulfilled the established CD diagnostic criteria (mean age: 6.6 years; mediana: 5 years). A typical CD presentation was observed for 66/103 ( 64 .1% ) children vs atypical 37/103 (35.9%). The levels of tTG antibody were correlated significantly with Marsh types in the entire population by Spearman rank correlation (r = 0.661, P < 0.0001), and separately for typical(r = 0.633, P < 0.001) and atypical (r = 0.574, P < 0.0001) groups. Histopathological evaluation of intestinal biopsies revealed total or subtotal VA in 44 patients (66.6%) in the typical group, and 24 patients (64.8%) in the atypical group. Partial VA was observed in 5 patients (7.5%) in the typical group and 1 (2.7%) patients in atypical group. There was no correlation of mode of presentation (typical and atypical) with the degree of villous atrophy by a non-parametric Mann-Whitney U test Z = ­ -1.437, P = 0.151. Conclusion: There is a positive correlation between tTG antibody serum level and duodenal histopathology in typical as in atypical pediatric CD. The degree of villous atrophy did not correlate with the mode of presentation, indicating that factors other than the degree of villous atrophy must account for typical simptoms in coeliac disease. doi: http://dx.doi.org/10.4021/ijcp57w

[1]  A. López-Vázquez,et al.  Differences between pediatric and adult celiac disease. , 2011, Revista espanola de enfermedades digestivas : organo oficial de la Sociedad Espanola de Patologia Digestiva.

[2]  M. Vieth,et al.  [Atypical celiac disease in a patient with type 1 diabetes mellitus and Hashimoto's thyreoiditis]. , 2011, Deutsche medizinische Wochenschrift.

[3]  A. Kalaycı,et al.  Celiac disease in 87 children with typical and atypical symptoms in Black Sea region of Turkey , 2009, World journal of pediatrics : WJP.

[4]  S. Neuhausen,et al.  Strongly Positive Tissue Transglutaminase Antibodies are Associated With Marsh 3 Histopathology in Adult and Pediatric Celiac Disease , 2008, Journal of clinical gastroenterology.

[5]  J. Barkin.  Correlation of Duodenal Histology With Tissue Transglutaminase and Endomysial Antibody Levels in Pediatric Celiac Disease , 2008 .

[6]  G. Bhagat,et al.  Lack of correlation of degree of villous atrophy with severity of clinical presentation of coeliac disease. , 2007, Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver.

[7]  C. Mitton,et al.  Can Tissue Transglutaminase Antibody Titers Replace Small-Bowel Biopsy to Diagnose Celiac Disease in Select Pediatric Populations? , 2005, Pediatrics.

[8]  F. Mascart,et al.  Antiendomysial and antihuman recombinant tissue transglutaminase antibodies in the diagnosis of coeliac disease: a biopsy-proven European multicentre study , 2005, European journal of gastroenterology & hepatology.

[9]  I. Korponay-Szabó,et al.  In vivo targeting of intestinal and extraintestinal transglutaminase 2 by coeliac autoantibodies , 2004, Gut.

[10]  D. Schuppan,et al.  The role of the immune response against tissue transglutaminase in the pathogenesis of coeliac disease. , 2004, Autoimmunity reviews.

[11]  A. Tursi,et al.  Prevalence of Antitissue Transglutaminase Antibodies in Different Degrees of Intestinal Damage in Celiac Disease , 2003, Journal of clinical gastroenterology.

[12]  D. Kelly,et al.  Metabolic liver disease: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition. , 2002 .

[13]  R. Troncone,et al.  Celiac Disease: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition , 2002, Journal of pediatric gastroenterology and nutrition.

[14]  F. Paparo,et al.  Anti-tissue transglutaminase antibodies from coeliac patients inhibit transglutaminase activity both in vitro and in situ , 2002, Gut.

[15]  L. Klareskog,et al.  Recombinant Human Tissue Transglutaminase for Diagnosis and Follow-Up of Childhood Coeliac Disease , 2002, Pediatric Research.

[16]  G. Holmes.  Potential and latent coeliac disease , 2001, European journal of gastroenterology & hepatology.

[17]  C. Catassi,et al.  The serum IgA class anti-tissue transglutaminase antibodies in the diagnosis and follow up of coeliac disease. Results of an international multi-centre study , 2001, European journal of gastroenterology & hepatology.

[18]  A. Torsoli,et al.  Identification of a new coeliac disease subgroup: antiendomysial and anti‐transglutaminase antibodies of IgG class in the absence of selective IgA deficiency , 2001, Journal of internal medicine.

[19]  D. Schuppan,et al.  Current concepts of celiac disease pathogenesis. , 2000, Gastroenterology.

[20]  M. Mäki,et al.  Coeliac disease , 1998, The Lancet.

[21]  L. Greco,et al.  Latent and potential coeliac disease , 1996, Acta paediatrica (Oslo, Norway : 1992). Supplement.

[22]  M. Marsh,et al.  Morphology of the mucosal lesion in gluten sensitivity. , 1995, Bailliere's clinical gastroenterology.

[23]  A. Ferguson,et al.  Intestinal antibody pattern of coeliac disease: association with gamma/delta T cell receptor expression by intraepithelial lymphocytes, and other indices of potential coeliac disease. , 1994, Gut.

[24]  M. Mäki,et al.  Follow-up of patients positive in reticulin and gliadin antibody tests with normal small-bowel biopsy findings. , 1993, Scandinavian journal of gastroenterology.

[25]  A. Ferguson,et al.  Clinical and pathological spectrum of coeliac disease--active, silent, latent, potential. , 1993, Gut.