Celiac disease (CD) is an immune-mediated disease typically associated with gastrointestinal inflammation in response to gluten exposure.
Esophagogastroduodenoscopy (EGD) with
duodenal biopsies has been considered “gold standard” for CD diagnosis; however, recent pediatric guidelines such as the European Society for Paediatric Gastroenterology Hepatology and Nutrition Guidelines for the Diagnosis of Coeliac Disease (https://journals.lww.com/jpgn/ Fulltext/2012/01000/European_Society_for_Pediatric_Gastroenterology,.28.aspx) suggest that serology screening may be an adequate substitute for EGD with duodenal biopsy. In particular, a tissue transglutaminase IgA antibody (TTG IgA) level greater than 10 times the upper limit of the measured laboratory value has a high association with CD. It is unclear if the degree of villi damage seen in CD corresponds well with TTG IgA antibody titer levels, and the authors of this study evaluated the effectiveness of determining intestinal villi length (disease severity) in pediatric patients with a new diagnosis of CD in regards to their long-term health outcomes as adults.
This study occurred at a single tertiary hospital in Finland, and retrospective data was obtained from a CD database containing the records of 906 pediatric patients from 1966 to 2014. Medical data at the time of CD diagnosis was reviewed such as the presence of gastrointestinal symptoms and other extra-intestinal disease manifestations, and duodenal biopsy results from these patients were divided into three groups: partial atrophy, subtotal atrophy, and total atrophy. Growth impairment was determined by decreased growth velocity noted on growth charts. TTG IgA and endomysial antibodies (EMA) were recorded from patients from 2000 onward when such serum testing had become available. Finally, pediatric patients with CD who were now adults completed three questionnaires including a questionnaire reviewing complications and co-morbidities associated with CD, the Gastrointestinal Symptom Rating Scale (GSRS) to evaluate the presence of gastrointestinal symptoms, and the Psychological General WellBeing questionnaire (PGWB) to evaluate quality of life.\
The duodenal biopsies of the 906 pediatric patients demonstrated partial villous atrophy in 34%, subtotal villous atrophy in 40%, and total
villous atrophy in 26% of patients. Children with total villous atrophy had significantly more extraintestinal manifestations, anemia, and impaired growth while patients with less severe atrophy had less abdominal pain and less CD detected by serum screening. Children with more advanced stages of villous atrophy were diagnosed during the earlier years of the study although there was no difference in patient age throughout the study at the time of CD diagnosis. More severe villous atrophy was identified in patients with significantly shorter height, lower body mass index (BMI), lower hemoglobin levels, and higher celiacantibody levels (TTG IgA and EMA) at time of CD diagnosis.
A total of 503 adult patients with CD diagnosed as children were asked to participate in this study, and 212 of these patients (42%) completed all questionnaires. The adult patients with partial and subtotal villous atrophy were significantly younger than the adult patients with total villous atrophy. However, all three villous atrophy groups had no difference as adults in regards to comorbid conditions, complications from celiac disease, selfreported symptoms, overall health, adherence to a gluten-free diet, GSRS score, and PGWB score even after adjusting for current age, sex, year of CD diagnosis, and median BMI.
This study demonstrates that pediatric patients with CD and more severe villous atrophy (and more health-related issues as children) appear to have similar long-term outcomes as adults when compared to pediatric patients with less severe villous damage. We can use this information to inform pediatric patients with CD and their families about the importance of continuing a gluten-free diet throughout their lives in order to have good health outcomes.
The duodenal biopsies of the 906 pediatric
patients demonstrated partial villous atrophy in 34%, subtotal villous atrophy in 40%, and total villous atrophy in 26% of patients. Children with total villous atrophy had significantly more extraintestinal manifestations, anemia, and impaired growth while patients with less severe atrophy had less abdominal pain and less CD detected by serum screening. Children with more advanced stages of villous atrophy were diagnosed during the earlier years of the study although there was no difference in patient age throughout the study at the time of CD diagnosis. More severe villous atrophy was identified in patients with significantly shorter height, lower body mass index (BMI), lower hemoglobin levels, and higher celiac
antibody levels (TTG IgA and EMA) at time of CD diagnosis.
A total of 503 adult patients with CD diagnosed as children were asked to participate in this study, and 212 of these patients (42%) completed all questionnaires. The adult patients with partial and subtotal villous atrophy were significantly younger than the adult patients with total villous atrophy. However, all three villous atrophy groups had no difference as adults in regards to comorbid conditions, complications from celiac disease, selfreported symptoms, overall health, adherence to a gluten-free diet, GSRS score, and PGWB score even after adjusting for current age, sex, year of CD diagnosis, and median BMI.
This study demonstrates that pediatric patients with CD and more severe villous atrophy (and more health-related issues as children) appear to have similar long-term outcomes as adults when compared to pediatric patients with less severe villous damage. We can use this information to inform pediatric patients with CD and their families about the importance of continuing a gluten-free diet throughout their lives in order to have good health outcomes.
Kroger S, Kurppa K, Repo M, Huhtala H, Kaukinen K, Lindfors K, Arvola T, Kivela L. Severity of villous atrophy at diagnosis in childhood does not predict long-term outcomes in celiac disease. Journal of Pediatric Gastroenterology and Nutrition 2020; 71: 71-77.