Celiac disease and Budchiari syndrome

Celiac disease is an immune-mediated enteropathy, caused (in genetically predisposed or susceptible individuals) by the ingestion of gluten, the complex of waterinsoluble proteins found in cereal grains such as wheat, rye and barley. In terms of terminology, it is the complex natural history and extremely polymorphous clinical presentation that has created some confusion. In fact, to date, at least three different forms of celiac disease are known, in addition to the simple non-celiac gluten sensitivity, since in common clinical practice most patients do not present the classic symptoms such as malabsorptive syndrome with diarrhoea, steatorrhoea, weight loss and nutritional defi ciency, but rather an anaemia, asthenia, meteorism, abdominal tension, osteoporosis and infertility, thus painting an extremely varied and complex symptomatic picture that is linked to enteric microbiota and microbiome issues. Celiac disease affects the mucosa of the small intestine, while it generally spares the submucosa, muscolaris propria and serosa; if the disease does not involve the whole of the small intestine but only part of it, it is usually more serious in the proximal than the distal tract. The simultaneous presence of shortened villi, crypt hyperplasia, the abnormal cytological appearance of the absorbent surface and increased lamina propria cells is required for the diagnosis of celiac disease. Based on these fi ndings, several forms of celiac disease have been identifi ed in the clinic: typical, atypical (and in turn silent, latent, and potential), and sensitive non-celiac. Based on these considerations a specifi c diagnostic scheme is suggested to frame the celiac universe more functionally and structurally (so-called Diagnostic Scheme for Celiac Disease and Nonceliac Gluten Sensitivity, DSCNC), identifying at least eight clinical hypotheses based on the serological, genetic, bioptic and allergological tests suggested). From a pharmacological and integrative point of view, the protocols shared by the scientifi c community remain in place: gluten-free diet, vitamin and salt supplementation if appropriate, pharmacological therapy (antibiotics, antihistamines, corticosteroids and immunosuppressants) if necessary, also in the future with the majority orientation oriented towards oral glutenase able to counteract the effects of gliadin in sensitive subjects, the use of larazotide acetate to remedy the increase in intestinal permeability and tTG inhibitors to reduce the toxic effects of gluten intake. The state of the art on celiac disease is not yet able to explain the precise aetiology and atypical forms of the disease, as well as the real impact of genetic predisposition on clinical manifestations. Research continues and seems to point the way to a complete resolution of this enteropathy that has been so prevalent over the last two decades.