The Spectrum of Celiac Disease: Epidemiology, Clinical Aspects and Treatment

Greetje J. Tack; Wieke H. M. Verbeek; Marco W. J. Schreurs; Chris J. J. Mulder

Disclosures
In This Article

Abstract and Introduction

Abstract

Celiac disease is a gluten-sensitive enteropathy that affects people of all ages worldwide. This disease has emerged as a major health-care problem, as advances in diagnostic and screening methods have revealed its global prevalence. Environmental factors such as gluten introduction at childhood, infectious agents and socioeconomic features, as well as the presence of HLA-DQ2 and/or HLA-DQ8 haplotypes or genetic variations in several non-HLA genes contribute to the development of celiac disease. Growing insight into the variable clinical and histopathological presentation features of this disease has opened new perspectives for future research. A strict life-long gluten-free diet is the only safe and efficient available treatment, yet it results in a social burden. Alternative treatment modalities focus on modification of dietary components, enzymatic degradation of gluten, inhibition of intestinal permeability and modulation of the immune response. A small group of patients with celiac disease (2–5%), however, fail to improve clinically and histologically upon elimination of dietary gluten. This complication is referred to as refractory celiac disease, and imposes a serious risk of developing a virtually lethal enteropathy-associated T-cell lymphoma.

Introduction

Celiac disease is the most common food intolerance in the Western population, and currently represents a major health-care issue. Celiac disease has an ancient history, first described in the 1st and 2nd century AD. In 1887, Samuel Gee described typical symptoms of celiac disease in children, including irritability, chronic diarrhea and failure to thrive, and cure by means of a diet was suggested for the first time.[1] Since then, insight into celiac disease has undergone a revolutionary development regarding epidemiology, diagnostics and treatment.

Celiac disease is a chronic, small-intestinal enteropathy, which is triggered by gluten proteins from wheat, barley and rye. Celiac disease is characterized by an autoimmune response in genetically susceptible individuals resulting in small-intestinal mucosal injury. As a consequence, malabsorption develops, which results in malnutrition-related problems including anemia, vitamin deficiencies, osteoporosis, and neurological disorders. Withdrawal of dietary gluten usually leads to prompt healing of the damaged small-intestinal mucosa and improvement of nutrient absorption. A gluten-free diet is sufficient to treat the overwhelming majority of patients with celiac disease and clinical improvement is usually evident within a few weeks.[2]

A small percentage (2–5%) of patients with adult-onset celiac disease, especially those diagnosed above the age of 50, does not respond to a gluten-free diet and is seen as suffering from refractory celiac disease (RCD). The occurrence of an enteropathy-associated T-cell lymphoma (EATL) is the major complication associated with RCD and is the main cause of death in this patient group.[3,4] Early identification of patients with RCD enables early intervention, which results in reduction in morbidity and mortality.[4,5]

This Review gives an overview of the latest trends in epidemiology, clinical course, diagnostics, complications and treatment with respect to the spectrum of celiac disease.

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