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Ulcerative Coeliac Disease
The main feature of Coeliac disease or coeliac sprue is a mucosal damage in the small bowel due to dietary exposition to prolamins in wheat, rye and barley in individuals with a genetic susceptibility. The treatment of this disease is straightforward - a lifelong gluten-free diet. This treatment will most often cure the disease and diminish the risk for any sequelaes.
Ulcerative lesions in the proximal part of the descending duodenum associated with coeliac sprue
In a small group of patients the glutenfree diet does not cure the abnormalities associated with coeliac disease. These patients are mostly older and the have suffered from symptoms due to coeliac disease for years before the diagnose is made. This form of coeliac disase has several ill-defined names. The name most often used is "Refractory coeliac disease". A typical feature of this condition is ulcerative lesions in the jejunum and ileum, and the names "Ulcerative jejunitis" or "Ulcerative jejunoileitis" are therefore used. The images illustrating this text are taken in the descending part of the duodenum, so these ulcerations can occur also in the proximal part of the small bowel.
Ulcerative lesions in the distal part of the descending duodenum associated with coeliac sprue
The ulcerations in the small bowel look benign, but they usually have a less favourable course, causing bleedings and perforations making surgical resection necessary. Medical treatment is not often effective. This condition is often associated with a synchronous or metachronous development of a T-cell lymphoma in the bowel or in other body sites. The term "Enteropathy associated T cell lymphoma (EATL)" is used for this condition. In most instances, EATL and Ulcerative jejunitis is the same condition.
Ulcerative lesions associated with coeliac sprue. The typical mucosal pattern in coeliac sprue is clearly seen in the image to the right.
We would like to suggest the name "Ulcerative coeliac disease" as a name for this severe condition. This name implies the most important etiology of this semimalignant or malignant sequelae of coeliac sprue, and as this disease obviouslu can occur also in the proximal part of the small bowel, this name is superior to e.g. ulcerative jejunoileitis.
The main features of this condition are:
- coeliac disease, which has not been treated because the diagnose has never been made before the ulcerative manifestations cause symptoms
- ulcerative lesions that endoscopically look benign and which can occur anywhere in the small bowel
- nonresponsiveness to glutenfree diet
- nonresponsiveness to normal treatment of duodenal ulcer disease
- nonresponsiveness to other conventional treatments including corticosteroids, making the prognosis poor in many instances
- a clear association with T cell lymphoma, making this condition a premalignant and in many instances a malignant condition
Ulcerative lesions associated with coeliac sprue
Some references concerning refractory coeliac disesase:
Biagi F, Corazza GR. Defining gluten refractory entropathy. Eur J Gastroenterol Hepatol 2001 May;13(5):561-565
Mulder CJ, Wahab PJ, Moshaver B, Meijer JW. Refractory coeliac disease: a window between coeliac disease and enteropathy associated T cell lymphoma. Scand J Gastroenterol Suppl 2000;(232):32-37
Ryan MB. Refractory Celiac Disease. Gastroenterology 2000; 119:243-251