Doença celíaca refratária

Coeliac disease has a complex pathogenesis that results from the interaction between environmental (dietary exposure to gluten), genetic and immunologic factors. Gluten-free diet induces clinical and histological recovery in the majority of patients. However, a proportion of patients does not respond to a glutenfree diet and will be diagnosed as Refractory Coeliac Disease (RCD). RCD can be classified in type 1, characterized by polyclonal intraepithelial lymphocytes with a normal immune phenotype, and type 2, characterized by monoclonal intraepithelial lymphocytes with an aberrant immune phenotype. RCD is associated with a high risk of complications such as malnutrition, lymphocytic gastritis, ulcerative jejunitis and enteropathy-associated T-cell lymphoma, especially type 2. The prognosis is poor in RCD type 2, with a 5-year survival of approximately 50%. RCD can be considered a precursor of lymphoma. The treatment of RCD, mainly type 2, remains a clinical challenge.