OBJECTIVE: The goal was to evaluate the impact of immunoglobulin A endomysial antibody testing on the incidence and clinical presentation of childhood celiac disease.
METHODS: The incidence and clinical presentation of celiac disease in patients <18 years of age in 1990–1996 (pretesting group) versus 2000–2006 (testing group) were compared.
RESULTS: The median age at diagnosis was 2 years (95% confidence interval: 2–4 years) in the pretesting group (N = 36), compared with 9 years (95% confidence interval: 8–10 years) in the testing group (N = 199; P < .001); the female/male ratios (1.6:1) were similar (P = .982). The incidence of celiac disease increased from 2.0 cases per 100000 children (pretesting group) to 7.3 cases per 100000 children (testing group; P = .0256). The frequency of classic celiac disease presentations decreased from 67% (pretesting group) to 19% (testing group; P < .001), but the incidence of classic celiac disease did not differ (0.8 vs 1.6 cases per 100000; P = .154). In the testing group, 13 previously unrecognized clinical presentations were observed in 98 children, including 35 with family history, 18 with abdominal pain, and 14 with type 1 diabetes mellitus. The frequency of Marsh IIIc lesions decreased from 64% (pretesting group) to 44% (testing group; P = .0403). In the testing group, classic celiac disease remained predominant (67%) in young children (<3 years), whereas atypical gastrointestinal and silent presentations predominated in older children.
CONCLUSIONS: Antibody testing for celiac disease tripled the incidence of celiac disease and quadrupled the median age at diagnosis.
Comments
Dermatitis herpetiformis is the specific cutaneous manifestation of gluten sensitive enteropathy
We read with interest the manuscript of McGowan et al (1) about the impact of serological testing on childhood celiac disease (CD), and we agree with the conclusions.
However, we have a criticism that should be pointed out. In the introduction, the Authors recommended serologic testing for CD in a wide variety of ailments, such as gastrointestinal symptoms, chronic fatigue, short stature, delayed puberty, dental enamel defects, elevated liver transaminase levels, dermatitis herpetiformis (DH), and nutritional anemias, as already suggested in current North American guidelines (2). Although correct for all the other disease listed, their recommendation is not valid for DH, that should be considered per se as a diagnostic tool to detect CD.
DH is a pruritic polymorphous skin disease that is now considered as the specific cutaneous manifestation of CD (3). The diagnostic gold standard for DH is direct immunofluorescence assay on healthy perilesional skin, that shows granular immunoglobulin A deposits at the dermoepidermal junction in almost all the patients. Clinical examination, skin histopathology and dosage of antibodies to transglutaminase achieve a definite diagnosis in the remaining cases (3).
Since DH is invariably associated with CD and the lifelong gluten- free diet is an effective treatment of both disease, DH is considered a specific manifestation of gluten sensitive enteropathy and the National Institute of Health stated that a duodenal biopsy is unnecessary for the diagnosis in CD patients with a proven DH (4). However, this view is not followed by the vast majority of the gastroenterologist. Therefore, we would like to recommend physicians dealing with CD to carefully investigate cutaneous signs and to take DH more frequently into account.
References
1. McGowan KE, Castiglione DA, Butzner JD. The changing face of childhood celiac disease in North America: impact of serological testing. Pediatrics 2009; 124: 1572-1578.
2. Hill ID, Dirks MH, Liptak GS, et al. Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2005; 40: 1–19.
3. Caproni M, Antiga E, Melani L, Fabbri P. Guidelines for the diagnosis and treatment of dermatitis herpetiformis. J Eur Acad Dermatol Venereol 2009; 23: 633-638.
4. National Institutes of Health Consensus Development Conference Statement on Celiac Disease, June 28-30, 2004. Gastroenterology 2005; 128: S1-9.
Conflict of Interest:
None declared