Criteria
Subgroups of coeliac disease
Common subgroups include:[129]
Classic coeliac disease: clinical symptoms and signs of malabsorption, including diarrhoea, steatorrhoea, weight loss or growth failure, abdominal pain and discomfort, and fatigue. Classic symptoms are found in <50% of patients.
Non-classical coeliac disease: lacks the typical gastrointestinal symptoms of malabsorption; presents with other gastrointestinal symptoms, deficiency states (e.g., iron deficiency), or extra-intestinal manifestations (e.g., fatigue, elevated liver enzymes, or infertility). Atypical disease likely accounts for the largest proportion of patients with a diagnosis of coeliac disease.
Asymptomatic coeliac disease: serological and histological evidence of coeliac disease but without any evident symptoms, signs, or deficiency states. The proportion of coeliac disease that is truly asymptomatic is not well known, but it is thought to account for at least 20% of patients.
Non-responsive coeliac disease: clinical symptoms or laboratory abnormalities typical of coeliac 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 coeliac disease: subtype of non-responsive coeliac disease. Persistence of clinical symptoms and signs with histological 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 coeliac disease diagnosis were in fact exposed to gluten.[130] The proportion of refractory coeliac disease among patients with coeliac disease is not precisely established, especially since refractory celiac 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 coeliac disease.
Marsh criteria[78]
Histological changes on small intestinal biopsy
0: normal villous architecture with no increase in intra-epithelial lymphocytes
I: normal villous architecture with increased intra-epithelial lymphocytes
II: increased intra-epithelial lymphocytes and crypt hyperplasia with normal villi
IIIa: increased intra-epithelial lymphocytes and crypt hyperplasia with partial villous atrophy
IIIb: increased intra-epithelial lymphocytes and crypt hyperplasia with subtotal villous atrophy
IIIc: increased intra-epithelial lymphocytes and crypt hyperplasia with total villous atrophy.
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