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Celiac Disease Complications

The complications of celiac disease include cancers, small bowel ulcers (ulcerative jejunoileitis), and collagenous celiac disease.

Cancer Linked to Celiac Disease

Adults with celiac disease have a several-fold higher than normal risk of developing lymphomas (cancers of the lymph glands) in the small intestine and elsewhere. They also have a high risk of small intestinal and, to a lesser degree, of esophageal carcinomas (cancers of the inner lining of the intestine and esophagus). Lymphoma tends to develop in adult patients who have had celiac disease for longer than 20-30 years and in patients with that don't respond to treatments for celiac disease. Symptoms -- if there are any -- of small intestinal lymphoma or carcinoma can include anemia, bleeding into the intestine, abdominal pain, weight loss, fever, and small intestinal obstruction (with symptoms of abdominal distension, vomiting, and cramping abdominal pain). Small intestinal lymphoma and carcinoma are difficult to diagnose. Sometimes in patients with celiac disease, where the disease has been controlled with a gluten-free diet, recurrence of weight loss, anemia, abnormal pain, and symptoms of intestinal obstruction will lead doctors to search for intestinal lymphoma and carcinoma.

Computerized tomography (CT) scans of the abdomen, enteroclysis (one type of barium X-ray of the small intestine), and enteroscopy (inspection of the small intestine using a long, flexible endoscope) are procedures doctors use to diagnose small intestinal lymphoma and carcinoma. Sometimes diagnoses of intestinal lymphoma or carcinoma can only be made with surgery (open laparotomy) or by laparoscopy (an exam of the abdominal cavity with flexible endoscopes). The prognosis for patients who develop intestinal lymphoma usually is poor. Long-term survival (survival beyond five years) of patients with small intestinal lymphoma is estimated to be only 10%. Other cancers more likely to occur in people with celiac disease include cancers of the liver, oral cavity, and large intestine.

Ulcerative Jejunoileitis

Ulcerative jejunoileitis is a rare complication of celiac disease. In ulcerative jejunoileitis there are recurrent episodes of small intestinal ulcerations and formation of strictures (narrowing of the intestinal lumen). Small intestinal ulcerations and stricture formation can lead to intestinal bleeding, weight loss, abdominal pain, and intestinal obstruction. Patients with ulcerative jejunoileitis are at high risk of developing intestinal lymphomas. The diagnosis of ulcerative jejunoileitis is made by enteroclysis of the small intestine, enteroscopy, or CT scan of the abdomen. Treatment involves a gluten-free diet and surgical removal of the most diseased portions of the small intestine. The prognosis is poor; long-term survival for patients with ulcerative jejunoileitis beyond five years is less than 50%.

Collagenous Celiac Disease

Collagenous celiac disease is a rare, but serious complication of celiac disease in which a patient may have the symptoms of celiac disease initially, but they fail to improve on a gluten-free diet, and after several years a large amount of scar tissue (collagen) forms just under the intestinal lining. There is no treatment for collagenous celiac disease, and the prognosis is poor.

Reducing Cancer Risk With Celiac Disease

Some doctors believe that strict adherence to a gluten-free diet can reduce the risk of cancer in patients with celiac disease, but further studies are needed to prove this. Until more is known in this area, patients with celiac disease should adhere strictly to a gluten-free diet.

WebMD Medical Reference

Reviewed by Andrew Seibert, MD on October 10, 2011

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