Major criteria:
(1) recurrence or persistence of symptoms and intestinal damage after gluten exclusion for at least 6 to 12 months
(2) exclusion of other causes of nonresponsiveness including gluten exposure
(3) need for an alternative therapy due to lack of response to gluten-free diet
(4) absence of overt systemic or intestinal lymphoma
(5) previous diagnosis of biopsy-proven celiac disease with history of clinical response to gluten-free diet.
(6) subtype based on aberrant/monoclonal T-cell intra-epithelial lymphocytes (RCD I if absent, RCD II if present)
where:
• Symptoms include diarrhea, involuntary weight loss, abdominal pain.
• Intestinal damage includes partial or complete villous atrophy on biopsy.
• Findings supportive for the diagnosis of celiac disease include: (1) family history of celiac disease, (2) HLA-DQ2 or HLA-DQ8, (3) positive serolologic celiac tests listed under minor criteria.
• T-cell phenotype determined by immunohistochemistry or T-cell clonality analysis. Aberrant clones (1) lack expression of the beta-chain of the T-cell receptor, CD3 and CD8, (2) express intracytoplasmic CD3, (3) have T-cell receptor gamma rearrangement by PCR or Southern blot.
Minor criteria:
(1) serologic findings: endomysial autoantibodies (EMA) or tissue transglutaminase autoantibodies (tTGA)
(1a) positive results support the diagnosis of celiac disease
(1b) negative results support compliance to gluten-free diet
(2) absence of anti-enterocyte antibodies
Interpretation:
• A definitive diagnosis requires the presence of all six major criteria. However the sixth criteria is used for subtyping.
