Source

Center for Celiac Research, University of Maryland School of Medicine, 22 S Greene St, N5W70, PO Box 140, Baltimore, MD 21201-1595, USA. afasano@umaryland.edu

Abstract

BACKGROUND:

Celiac disease (CD) is an immune-mediated enteropathic condition triggered in genetically susceptible individuals by the ingestion of gluten. Although common in Europe, CD is thought to be rare in the United States, where there are no large epidemiologic studies of its prevalence. The aim of this study was to determine the prevalence of CD in at-risk and not-at-risk groups in the United States.

METHODS:

Serum antigliadin antibodies and anti-endomysial antibodies (EMA) were measured. In EMA-positive subjects, human tissue transglutaminase IgA antibodies and CD-associated human leukocyte antigen DQ2/DQ8 haplotypes were determined. Intestinal biopsy was recommended and performed whenever possible for all EMA-positive subjects. A total of 13 145 subjects were screened: 4508 first-degree and 1275 second-degree relatives of patients with biopsy-proven CD, 3236 symptomatic patients (with either gastrointestinal symptoms or a disorder associated with CD), and 4126 not-at-risk individuals.

RESULTS:

In at-risk groups, the prevalence of CD was 1:22 in first-degree relatives, 1:39 in second-degree relatives, and 1:56 in symptomatic patients. The overall prevalence of CD in not-at-risk groups was 1:133. All the EMA-positive subjects who underwent intestinal biopsy had lesions consistent with CD.

CONCLUSIONS:

Our results suggest that CD occurs frequently not only in patients with gastrointestinal symptoms, but also in first- and second-degree relatives and patients with numerous common disorders even in the absence of gastrointestinal symptoms. The prevalence of CD in symptomatic patients and not-at-risk subjects was similar to that reported in Europe. Celiac disease appears to be a more common but neglected disorder than has generally been recognized in the United States.

Source

Center for Celiac Research, Division of Pediatric Gastroenterology and Nutrition, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA.

Abstract

PURPOSE OF REVIEW:

Celiac disease is a syndrome characterized by damage of the small intestinal mucosa caused by the gluten fraction of wheat proteins and similar alcohol-soluble proteins (prolamines) of barley and rye in genetically susceptible subjects. The presence of gluten in these subjects leads to a self- perpetuating mucosal damage, and the elimination of gluten results in full mucosal recovery. The clinical manifestations of celiac disease are protean in nature and vary markedly with the age of the patient, the duration and extent of disease, and the presence of extraintestinal pathologic changes. In addition to the classic gastrointestinal form, a variety of other clinical manifestations of the disease have been described, including atypical and asymptomatic forms. Although the typical form of celiac disease, characterized by failure to thrive, is still the most frequent presentation in the pediatric age group, severe growth delay is less commonly seen in developed countries.

RECENT FINDINGS:

Recent epidemiologic studies suggest that celiac disease-associated growth retardation is becoming a tangible health problem in developing countries, where the problem has been historically overlooked. Given the protean nature of the clinical presentation of celiac disease, the diagnosis is extremely challenging and relies on a sensitive and specific algorithm that allows the identification of different manifestations of the disease. Serologic tests developed in the past decade provide a noninvasive tool for screening individuals at risk for the disease as well as the general population.

SUMMARY:

The current gold standard for the diagnosis of celiac disease remains histologic confirmation of the intestinal damage in serologically positive individuals. The keystone treatment of celiac disease patients is a lifelong elimination diet in which food products containing gluten are avoided.