Case series of Cryptogenic Multifocal Ulcerating Stenosing Enteritis (CMUSE)

Background and study aims : Small bowel ulceration poses a limited, but difficult differential diagnosis. The most common causes are Crohn's disease (CD), NSAID-associated enteritis, lymphoma, cytomegaly virus infection and tuberculosis. A less known and relatively novel differential diagnosis is cryptogenic multifocal ulcerative stenosing enteritis (CMUSE). Patients and 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 in order to increase general knowledge on CMUSE. Results : Five patients, 3 males and 2 females, with a mean age of 39 +/- 5 years, underwent balloon-assisted enteroscopy. Typical short, circular, ulcerative stenoses were detected in the jejunum in 2 and in the ileum in 3 patients. The number of stenoses ranged from 1 to 7 per patient. Histopathology revealed nonspecific granulocyte inflammation without specific CD findings. Stenoses were often missed on pre-enteroscopy CT or MRI enteroclysis due to their short length. Treatment consisted of endoscopic balloon dilation in 3, corticosteroids in 3, azathioprine in 1 and anti-TNF alpha biologicals in 3 patients. 3 patients needed additional surgery because of ongoing symptomatic small bowel stenosis or retained wireless videocapsule. Conclusion: In patients with short, ulcerative small intestinal stenoses CMUSE is an important but often neglected differential diagnosis. The pathophysiology and relationship to CD are subject of ongoing debate, but specific endoscopic characteristics, different histopathological findings and lack of clear abnormalities on CT or MRI enterography suggest that CMUSE is a distinct albeit rare chronic inflammatory bowel disease.