Review and feature article
Prototypic disorders of gastrointestinal mucosal immune function: Celiac disease and Crohn's disease

https://doi.org/10.1016/j.jaci.2004.11.011Get rights and content

Celiac disease is a disorder of the small intestine characterized by chronic inflammation of the mucosa and protean clinical manifestations caused by loss of tolerance to dietary antigens. Two strongly associated cofactors have been identified: the presence of HLA-DQ2 or HLA-DQ8 in the host and specific antigenic peptides in the diet that are present in wheat, rye, and barley. Most patients have complete remission after dietary elimination of these foods. Crohn's disease is characterized by chronic, relapsing, recurrent, focal, transmural inflammation of the gastrointestinal tract that can lead to multiple serious problems requiring chronic medical and surgical therapy. Crohn's disease is associated with multiple genetic mutations, at least one of which has been clearly implicated in innate immunity. Multiple lines of evidence suggest that the disease involves abnormal immune responses to gut microbial flora.

Section snippets

Overview

The mucosal immune system of the gastrointestinal tract provides the host with mechanisms to protect itself against invasion by potential pathogens and at the same time permits tolerance for potentially immunogenic products of digestion and the resident normal intestinal microbial flora. A number of important disorders of the gastrointestinal mucosa involve disturbances of these fundamental immunologic mechanisms. This article will describe 2 diseases for which there has been significant

Background

Celiac disease is a disorder of the small intestine characterized by chronic inflammation of the mucosa, which leads to atrophy of the intestinal villi, malabsorption, and protean clinical manifestations.1 It is also strongly associated with the skin disorder dermatitis herpetiformis. Both the major host genetic and immunologic risk factor, the presence of HLA-DQ2 and HLA-DQ8, and the necessary environmental factors, peptides in the diet that are present in wheat, rye, and barley, have been

Background

Crohn's disease is a syndrome of unknown cause characterized by chronic, recurrent, focal, transmural inflammation of the gastrointestinal tract in any location but most typically in the terminal ileum, colon, and small intestine. The disease typically has a relapsing and remitting course that could lead to multiple serious symptoms and complications, including strictures, perforation, and fistulae, which frequently require surgical therapy in addition to medical management.12

The disease is

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