Chapter 18

Celiac disease (CD) is “a permanent sensitivity to gluten in wheat and related proteins found in barley and rye, occurring in genetically susceptible individuals, and manifesting as an immune mediated enteropathy as defined by characteristic changes seen on intestinal histology.”1 A conservative definition requires the following:

• typical signs or symptoms;
• presence of CD-associated antibodies;
• a small intestinal biopsy showing villous atrophy;
• resolution of clinical manifestations with a gluten-free diet (GFD), including complete healing of the intestinal mucosa;
• reduction or disappearance of the CD-associated antibodies on a GFD.

In practice, it is questionable whether it is necessary to meet all aspects of this definition. Controversy continues about whether a small bowel biopsy is required to diagnose CD. Because of numerous reports of CD-associated seropositive individuals with no signs or symptoms of CD, guidelines continue to require a biopsy to confirm the diagnosis and need for treatment.

Different terms have been applied to common clinical situations:

• Classic CD refers to a presentation with typical clinical features such as diarrhea, abdominal pain, failure to thrive, or abdominal distention.
• Atypical CD describes a non-traditional presentation, primarily with extraintestinal manifestations, such as arthritis or iron deficiency anemia. In older children and adults, the atypical presentation may be more common than the “classic” presentation.
• Silent CD describes the situation of an individual without signs or symptoms of CD, but who has small bowel biopsy evidence of CD; usually these patients have an associated condition or a family history of CD, and are identified on screening as having CD-associated antibodies.
• Latent CD applies to individuals without signs or symptoms of CD, but who have some risk for future development of CD, such as expression of CD-related antibodies or DQ2 or DQ8 permissive genes, family history of CD, or having an associated condition. These individuals do not have small bowel biopsy changes, but may have CD-associated antibodies.
• Refractory CD describes an individual with defined CD who continues to have signs or symptoms of active CD despite pursuing a GFD. In this situation, considerations include considered exposure to gluten, enteropathy-associated T-cell lymphoma (EATL), or possibly another condition such as allergy or inflammatory bowel disease.

### Incidence and Prevalence

The prevalence in the United States and Europe is roughly 3–13 cases per 1000 individuals (1:300 to 1:80).1 There is a female predominance with a ratio of roughly 2:1.2 These estimates indicate that there are approximately 3 million people with CD in the United States alone, and a roughly equal number in Europe, of which 90% are undiagnosed (Table 18–1). Recent screening studies suggest that in developing countries in Africa, parts of Asia, and South America, the frequency is similar to that of the U.S. and European countries.3 To date, there are very little data exploring the rates of CD in China, Japan, and Southeast Asian countries, and these populations are thought to be ...

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