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Nomenclature for different phenotypes of celiac disease

Nomenclature for different phenotypes of celiac disease
Key characteristics Classic Non-gastrointestinal* Silent or subclinical Potential
Symptoms of malabsorption (eg, weight loss, steatorrhea, or signs of vitamin deficiency) + ±
Non-gastrointestinal symptoms (eg, liver disease, skin, arthritis, or neuropsychiatric) ± +
Positive celiac-specific antibody testΔ + + + +
Characteristic intestinal biopsy (villous atrophy) + + +
Mucosal healing and resolution of symptoms after beginning a gluten-free diet + + N/A N/A
N/A: not applicable; tTG: tissue transglutaminase; DGP: deamidated gliadin peptide; IgG: immunoglobulin G; IgA: immunoglobulin A.
* In the past, non-gastrointestinal celiac disease was termed "atypical" because it was thought to be uncommon, but the advent of serologic screening for celiac disease has identified a substantial proportion of patients with this pattern of symptoms.
¶ Skin lesions may include dermatitis herpetiformis. Many, but not all, patients with dermatitis herpetiformis have intestinal lesions consistent with celiac disease, with or without intestinal symptoms.
Δ The celiac-specific antibody tests are tTG, anti-endomysial antibodies, or DGP. Standard IgG- or IgA-anti-gliadin antibodies have low specificity and are not sufficient to support the diagnosis of celiac disease.
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