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Definition of different states of celiac disease. |
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| States of celiac disease (CD) |
Definition |
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| Clinically overt CD |
Typical gastrointestinal symptoms and signs of malabsorption. Histological changes with villous atrophy and hypertrophic crypts (Marsh type-3 lesion, see table 2). |
| Symptomatic (active) CD |
Same findings as in clinically overt CD |
| Silent CD |
Asymptomatic patients with typical histological changes (Marsh type-3) |
| Asymptomatic CD |
Same findings as in silent CD |
| Atypical CD |
Extraintestinal findings such as IgA-nephropathy and neurological symptoms. Typical histological changes. |
| Latent CD/potential CD |
Subjects with genetic predisposition who have initially a normal histology with no atrophy or crypt hyperplasia. Immunological abnormalities such as increased count of IELs (particularly gamma-delta T cells, Marsh type-1) and positive EMA or tTG-antibody tests are sometimes present. These subjects may develop clinically overt CD later in life. |
| Refractory CD |
Patients who do not respond to a gluten-free diet or who previously responded but later become non-responsive to a gluten-free diet. Intestinal lymphoma may have developed. Inadvertent gluten ingestion and other diseases must be excluded (see differential diagnosis). |
Holtmeier and Caspary Orphanet Journal of Rare Diseases 2006 1:3 doi:10.1186/1750-1172-1-3 |
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