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P248. Cryptogenic multifocal ulcerating stenosing enteritis (CMUSE): to be differentiated from Crohn's disease

H. Ooms, H. De Schepper, E. Macken, P. Pelckmans, T. Moreels, Antwerp University Hospital, Antwerp, Belgium, Gastroenterology & Hepatology, Edegem - Antwerp, Belgium

Background

Ulceration of the small bowel poses a rather limited but difficult differential diagnosis. The most common causes are Crohn's disease, NSAID-associated enteritis, lymphoma, CMV and tuberculous enteritis. An important and relatively novel differential diagnosis is CMUSE or cryptogenic multifocal ulcerative stenosing enteritis.

Methods

Five patients referred for balloon-assisted enteroscopy for various reasons showed endoscopic features of CMUSE. These findings and, when available, medical imaging were reviewed.

Results

Five patients, 3 males and 2 females, with a mean age of 39±5 years (range 28–54) underwent double- or single balloon enteroscopy. There was no history of NSAID use. Typical short circular ulcerative stenoses were detected in the jejunum in 2 and in the ileum in 3 patients without colonic abnormalities. The number of stenoses ranged from 3 to 7 per patient. Histopathological characterisation revealed nonspecific granulocyte inflammation without specific Crohn's disease findings in all cases. Due to the short length (<1 cm) of the circular stenoses, they were often missed on pre-enteroscopy CT or MRI enteroclysis. Treatment consisted of endoscopic balloon dilation up to 15 mm during enteroscopy in 3 patients, corticosteroids in 3, azathioprine in 1 and anti-TNFalfa biologicals in 3. Complete mucosal healing and disappearance of abdominal complaints occurred after induction therapy with infliximab. Nevertheless, 3 patients needed additional surgery because of ongoing symptomatic small bowel stenosis in 2 and in 1 because of a retained wireless video capsule at the level of a circular intestinal stenosis.

Conclusion

In patients with very short ulcerative small intestinal stenoses without signs of systemic inflammation, CMUSE is an important but often neglected differential diagnosis, with specific genetic findings. The pathophysiology and relationship to Crohn's disease are the subject of ongoing debate and controversy, but the specific endoscopic characteristics and the lack of clear abnormalities on CT or MRI enterography do suggest CMUSE is a distinct albeit rare chronic inflammatory bowel disease.