Cryptogenic multifocal ulcerous stenosing enteritis: a review of the literature

Abstract

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is an extremely rare illness characterised by chronic or relapsing subileus status resulting from multiple small intestinal fibrous strictures and multiple shallow ulcers of the small bowel. The etiology is unknown and pathogenesis is not fully understood. Therapy with systemic glucocorticosteroids is the treatment of choice. However, most patients develop corticosteroid dependence. Deep enteroscopy enables precise diagnostic work, possible endoscopic treatment of stenoses; may obviate the need for surgery and prevent excessive small bowel resections.

Figure 1

Double balloon enteroscopy. Severe inflammatory small intestinal involvement in front of tight stenosis of the jejunum.

Figure 2

Double balloon enteroscopy. A large but shallow ulcer is seen in front of tight fibrous stricture of the distal jejunum.

Figure 3

Increased content of collagen was found in interstitial tissue of the small bowel. Optical histology, hematoxylin-eosin staining. Original magnification 40x. Courtesy of Věra Tyčová, MD, the Fingerland Department of Pathology, Charles University, Faculty of Medicine and University Teaching Hospital, Hradec Králové.

Figure 4

Small intestinal epithelium, impaired by extensive infiltration with plasmatic cells and lymphocytes. Optical histology, haematoxilin-eosin staining. Original magnification 100x. Courtesy of Věra Tyčová, MD, the Fingerland Department of Pathology, Charles University, Faculty of Medicine and University Teaching Hospital, Hradec Králové.

Figure 5

Prevailing infiltration with plasmatic cells was identified in all layers of the small intestine. Immunohistochemistry, anti-CD138 staining. Original magnification 100x. Courtesy of Věra Tyčová, MD, the Fingerland Department of Pathology, Charles University, Faculty of Medicine and University Teaching Hospital, Hradec Králové.

Figure 6

Enteroclysis. Multiple persisting stenoses of the small intestine caused by fibrous strictures. Courtesy of Zdenĕk Vacek, MD, Department of Radiology, Charles University Faculty of Medicine and University Teaching Hospital, Hradec Králové.