Celiac sprue (review)

Celiac sprue may be defined as a model autoimmune disease with known trigger (gluten), a tight genetic linkage (with HLA-DQ2 and HLA-DQ8) and a specific humoral autoimmune response (autoantibodies to tissue transglutaminase, tTG). Gliadin peptides are repeatedly presented to HLA-DQ2 and HLA-DQ8 positive cells and induce an immune response in small-intestinal mucosa. tTG is a specific endomysial autoantigen released during cellular stress and by deamidation of gliadin peptides as well as by binding with them facilitates their interaction with HLA-DQ2 and HLA-DQ8 cells. CS is the consequence of an inappropriate by T-cells mediated immune reaction to gluten. The diagnosis is based on criteria of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition revised in 1990. The availability of sensitive and specific detection methods of serum antibodies to endomysium (AEA) and tTG (AtTGA) was followed by recognition of a broad spectrum of both the clinical presentation and histologic changes of intestinal mucosa in CS. The majority of patients have atypical clinical symptoms. The present prevalence of CS amounts to approximately 1:250. The following forms of CS are distinguished: classic (typical), latent, potential, subclinical, and silent. CS is frequently associated with other diseases and many of them are also of autoimmune origin. Serologic testing is indicated in subjects with atypical symptoms and autoimmune diseases. In cases with distinct suspicion biopsy should be always performed irrespective of the serologic tests. In refractory sprue the cryptic form of enteritis associated T-cell lymphoma should be excluded. Gluten-free diet is the cornerstone of CS therapy. The Alimentary Codex in individual countries admits different amounts of residual gluten in gluten-free products. In future years new basic knowledge as well as practical diagnostic and therapeutic recommendations may be expected.