Criteria
Subgroups of celiac disease
Common subgroups include:[129]
Classic celiac disease: clinical symptoms and signs of malabsorption, including diarrhea, steatorrhea, weight loss or growth failure, abdominal pain and discomfort, and fatigue. Classic symptoms are found in <50% of patients.
Nonclassical celiac disease: lacks the typical gastrointestinal symptoms of malabsorption; presents with other gastrointestinal symptoms, deficiency states (e.g., iron deficiency), or extraintestinal manifestations (e.g., fatigue, elevated liver enzymes, or infertility). Atypical disease likely accounts for the largest proportion of patients with a diagnosis of celiac disease.
Asymptomatic celiac disease: serologic and histologic evidence of celiac disease but without any evident symptoms, signs, or deficiency states. The proportion of celiac disease that is truly asymptomatic is not well known, but it is thought to account for at least 20% of patients.
Nonresponsive celiac disease: clinical symptoms or laboratory abnormalities typical of celiac disease fail to improve within 12 months of gluten withdrawal, or typical symptoms or laboratory abnormalities recur while the patient is on a gluten-free diet.
Refractory celiac disease: subtype of nonresponsive celiac disease. Persistence of clinical symptoms and signs with histologic abnormalities (villous atrophy) after at least 12 months on a strict gluten-free diet, and no evidence of another abnormality including overt lymphoma. Recent evidence confirmed that a proportion of individuals carrying a refractory celiac disease diagnosis were in fact exposed to gluten.[130] The proportion of refractory celiac disease among patients with celiac disease is not precisely established, especially since refractory coeliac disease type 1 does not have pathognomonic features and may be misclassified (gluten exposure, slow responders). The subtype refractory celiac disease 2 has precise diagnosis criteria of aberrant T cells and it involves less than 0.1% of patients with celiac disease.
Marsh criteria[79]
Histologic changes on small intestinal biopsy
0: normal villous architecture with no increase in intraepithelial lymphocytes
I: normal villous architecture with increased intraepithelial lymphocytes
II: increased intraepithelial lymphocytes and crypt hyperplasia with normal villi
IIIa: increased intraepithelial lymphocytes and crypt hyperplasia with partial villous atrophy
IIIb: increased intraepithelial lymphocytes and crypt hyperplasia with subtotal villous atrophy
IIIc: increased intraepithelial lymphocytes and crypt hyperplasia with total villous atrophy.
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