Multifocal stenosing ulceration of the small intestine

Abstract

Several reports have described an apparently uncommon clinicopathological disorder that is characterized by multifocal stenosing small-intestinal ulceration. Compared to Crohn's disease, the ulcers are not transmural and typically remain shallow, and involve only the mucosa and submucosa. The disorder seems to be localized in the jejunum and proximal ileum only, and not the distal ileum or colon. Only nonspecific inflammatory changes are present without giant cells or other typical features of granulomatous inflammation. Most patients present clinically with recurrent obstructive events that usually respond to steroids, surgical resection, or both. With the development of newer imaging modalities to visualize the small-intestinal mucosa, such as double-balloon enteroscopy, improved understanding of the long-term natural history of this apparently distinctive disorder should emerge.

Young woman with recurrent vomiting associated with weight loss

“…However, as in our patient, ongoing symptoms after resection are common. Association with mesenteric arterial microaneurysms (seen in this patient), venopathy or complement deficiency is reported 1 4 5. Although distinction from polyarteritis nodosa is sometimes difficult, patients with cryptogenic multifocal ulcerating and stenosing enteritis often lack many characteristic clinical features of polyarteritis nodosa such as those defined by the American College of Rheumatology 1.…”

“…Treatment is with steroids, but response is variable and many patients remain steroid dependent. Surgical resection is often required, which may result in complete recovery in up to 40% of patients 4. However, as in our patient, ongoing symptoms after resection are common.…”

Small Intestinal Multifocal Stenosing Ulceration

“…Several entities, shown in Table 1, have been associated with small bowel ulceration and subsequent stricture formation [8]. In ulcerative (non-granulomatous) jejunoileitis, moderate-to-severe architectural changes occur with flattening of the villi, often associated with celiac disease, or as a complication of celiac disease per se.…”

“…Crohn's disease, for example, may uncommonly occur in the proximal small bowel including either duodenum or jejunum without evidence of disease elsewhere [10], usually associated with other clinical and pathological features, as detailed in Table 2 [8]. At the same time, since precise diagnosis of Crohn's disease may be difficult, consideration of each of these entities, including CMUSE, is important to anticipating a clinical response to specific forms of therapy.…”

Ulcerating and stenosing enteropathy treated with misoprostol

“…Most intestinal ulcers in patients who do not have Crohn's disease are caused by NSAIDs, but idiopathic and nonspecific ulcers are also seen including a clinical condition called cryptogenic multifocal ulcerating stenosing enteropathy (CMUSE) described in case series [1][2][3]. The intestinal pathology of CMUSE is similar to NSAID enteropathy and a non-drug-related defect in prostaglandin (PG) biosynthesis or signalling is a possible pathogenic pathway.…”