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Celiac disease - refractory forms

Publication at First Faculty of Medicine |
2014

Abstract

Celiac disease is a hereditary autoimmune disorder, caused by a life-long gluten intolerance. Its only treatment is gluten-free diet, which, if followed properly, leads to disappearance of clinical symptoms and improved condition of the mucous membrane in the small intestine.

Refractory celiac disease is the condition where the celiac symptoms persist for more than a year even when gluten-free diet is followed. There are two types of refractory celiac disease, categorised depending on the character of the mucous membrane infiltration and the presence or non-presence of aberrant intra-epithelial lymphocytes.

Type I has positive prognosis and its course is similar to ordinary celiac disease. Type II on the other hand has high mortality and it can be seen as a cryptogenic, intraepithelial lymphoma with limited options for treatment.