Celiac sprue is a relatively rare chronic disease, in which there is a chracteristic mucosal lesion of the small intestine and impaired nutrient absorption which improves on withdrawal of wheat glidins and related grain proteins from the diet. This condition is known by a variety of names gluten sensitive enteropathy, nontropical sprue celiac disease. Celiac Sprue occurs largely in whites and is rare or nonexistent among native Africans, Japanese and Chinese

The fundamental disorder in celiac sprue is a sensitivity to gluten which is the alcohol soluble closely related graisn (oat, barley and rye) Cell mediated immunity appears to be important in the pathogenesis to gluten accumulates intraepithelial cytotoxic T cells and large numbers of lamina propria T helper cells that are thought to damage the intestinal entrocytes.

Although the genetic trends in this disease are variable a specific HLA Do?/? heterodimer appears to confer susceptibility through its interaction with glaidin. Ninety per cent to 95% of patients express the DQw2 histocompatibility antigen on chromosome 6, particularly a Do?/? disease. Because the DQ locus is linked with HLA B8 as many as 80% of patients express this latter antigen as well. An intriguing hypothesis invokes cross-reactivity of gliadin with a fragment of the E1b protein of type 12 adenovirus raising the possibility that celiac disease results in part from environmental exposure to this virus.

Clinical Features

The symptoms of celiac sprue vary tremendously from patient. Symptomatic diarrhea and failure to thrive may be evident during infancy, yet elders may seek attention only in their forties. The classic presentation includes diarrhea , flatulence, weight loss, and fatigue. Extraintestinal manifestations of malabsorption may overshadow the intestinal symptoms. Detection of circular overshadow the intestinal symptoms. Detection of circulating antigliadin or antiendomesial antibodies strongly favors the diagnosis. Definitive diagnosis rests on (1) clinical documentation of malabsorption (2) demonstration of the intestinal lesion by small bowel biopsy and (3) unequivocal improvement in both symptoms and mucosal histology on gluten withdrawal from the diet. If there is doubt about the diagnosis, gluten challenge followed by rebiopsy has been advocated.

Most patients with celiac sprue who adhere to a gluten free diet remain well indefinetly and ultimately die of unrelated causes. There is a long term risk of malignant disease, although it may be less than a twofold increase over the usual raate. More than half of these malignancies are intestinal lymphomas including a disproportionately high number T-cell lymphomas Other malignancies include gastrointestinal and breast carcinomas.