Celiac Disease

CD: Introduction (2006-2007)

Celiac Disease Evidence Analysis Project

 

Introduction

Celiac disease is a genetically-based autoimmune disease characterized by chronic inflammation of the small intestinal mucosa. Individuals with celiac disease have an immunologic reaction to specific sequences of amino acids found in the grains wheat, rye, and barley.

When these amino acid sequences are consumed by a person with celiac disease they trigger an immune system response that causes damage to the small intestinal mucosa. Inflammation and villous atrophy may lead to malabsorption of nutrients.

Celiac disease was once thought to be a rare childhood disease, but is now recognized as a fairly common multi-system disorder, occurring in one in 133 people (1). The only currently available treatment for celiac disease is life long adherence to a gluten-free meal pattern including strict avoidance of proteins from wheat, rye, and barley. Although the classic presentation of celiac disease is diarrhea, wasting, malabsorption, failure to grow, bloating, and abdominal cramps, not all individuals have symptoms. Recent studies have shown that many individuals with celiac disease are diagnosed when seeking medical care for other issues such as anemia, osteoporosis, peripheral neuropathy, infertility, and fatigue (2, 3).

Four main categories of celiac disease were included in The National Institutes of Health Consensus Development Conference Statement on Celiac Disease. They are as follows (4):

Classical celiac disease is dominated by symptoms and sequelae of gastrointestinal malabsorption. The diagnosis is established by serological testing, biopsy evidence of villous atrophy and improvement of symptoms on a gluten-free diet”

Celiac disease with atypical symptoms is characterized by few or no gastrointestinal symptoms, and extra intestinal manifestations predominate. Recognition of atypical features of celiac disease is responsible for much of the increased prevalence, and now may be the most common presentation. As with classical celiac disease, the diagnosis is established by serologic testing, biopsy evidence of villous atrophy, and improvement of symptoms on a gluten-free diet.

Silent celiac disease refers to individuals who are asymptomatic but have a positive serologic test and villous atrophy on biopsy. These individuals usually are detected via screening of high-risk individuals, or villous atrophy occasionally may be detected by endoscopy and biopsy conducted for another reason.

Latent celiac disease is defined by a positive serology but no villous atrophy on biopsy. These individuals are asymptomatic, but later may develop symptoms and/or histologic changes.

Dermatitis Herpetiformis (DH) is the skin manifestation of celiac disease. It is characterized by a bilateral, symmetric rash or eruptions primarily on pressure points of the skin that may evolve into blisters or bullae (fluid-filled sacs). These lesions are painfully itchy. Diagnosis is made through a skin biopsy taken from a site next to a lesion. While the vast majority of individuals with DH have no gastrointestinal symptoms characteristic of CD, most have biopsy-proven villous atrophy that responds well to a gluten-free diet. Topical treatment of the lesions with sulfa pyridine or Dapsone (along with a gluten-free diet) also is effective in treating this form of bullous atopic dermatitis.

Gluten sensitivity/intolerance is considered a non autoimmune response to the ingestion of gluten (4). Symptoms include, but are not limited to, abdominal pain and bloating, diarrhea, joint pain, reflux, bloating, and fatigue. Unlike celiac disease and dermatitis herpetiformis, gluten sensitivity does not cause atrophy of the intestinal villi.

As the only treatment for celiac disease and dermatitis herpetiformis (and possibly gluten sensitivity) is lifelong adherence to the gluten free diet, there must be consensus on what constitutes this particular diet. In addition to the obvious elimination of wheat, rye and barley, ingredients containing harmful proteins from these grains must be eliminated. Cross contamination with wheat, rye, and barley during food processing, preparation, and handling also must be avoided. We now know that ingestion of even a small amount of gluten can cause intestinal damage. Therefore careful monitoring of ingredients and processing is an intricate part of the gluten free diet.

References

  1. Green, PH. The many faces of celiac disease: clinical presentation of celiac disease in the adult population. Gastroenterology. 2005;128:S74-78.
  2. Brandimarte G, Tursi A, Giorgetti GM. Changing trends in clinical form of celiac disease. Which is now the main form of celiac disease in clinical practice? Minerva Gastroenterol Dietol. 2002;48:121-30.
  3. National Institutes of Health Consensus Development Program. Consensus Development Conference Statement. NIH Consensus Conference on Celiac Disease, June 28-30, 2004, http://consensus.nih.gov/2004/2004CeliacDisease118html.htm.
  4. Thompson, T. Celiac Disease versus Gluten Sensitivity. Available at: http://www.diet.com/dietblogs/read_blog.php?title=Celiac+Disease+vs.+Gluten+Sensitivity&blid=11838.

updated 1/14/09