Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type ofvasculitis or a disease mimicking vasculitis

Background/aims-Cryptogenetic multifocal ulcerous stenosing enteritis (CMUS E) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathop hysiology of the disease, and to propose a treatment strategy. Methods-A total of 220 French gastroenterology departments were contacted t o review patients with unexplained small bowel strictures. Of 17 responses, 12 corresponded to a diagnosis of CMUSE, These patients were hospitalised between 1965 and 1993 and their medical records were reviewed. Results-All patients (mean age 42.1 (4.4) years) had intestinal and five ha d extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca sy ndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One p atient had heterozygous type I C2 deficiency (28 base pair gene deletion). Two to 25 (mean 8.3 (1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on thr ee of five mesenteric angiograms. Despite surgery, symptoms recurred in sev en of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showe d superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils. Conclusions-CMUSE is an independent entity characterised by steroid sensiti ve inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly int estinal expression or with an as yet unclassified vasculitis.