title: Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis.
abstract: OBJECTIVE
  Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) 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 pathophysiology of the disease, and to propose a treatment strategy.
  
  
  METHODS
  A total of 220 French gastroenterology departments were contacted to 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 had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient 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 three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed 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 sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.
authors.affiliation: Service d'Hépato-gastroentérologie, Hôpital de Bicêtre, 78, rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.
authors.forename: G
authors.initials: G
authors.lastname: Perlemuter
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.forename: P
authors.initials: P
authors.lastname: Legman
authors.forename: L
authors.initials: L
authors.lastname: Weiss
authors.forename: D
authors.initials: D
authors.lastname: Couturier
authors.forename: S
authors.initials: S
authors.lastname: Chaussade
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003251:Constriction, Pathologic / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D003937:Diagnosis, Differential; D004381:Duodenal Ulcer / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D004751:Enteritis / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D013256:Steroids; D016896:Treatment Outcome; D014657:Vasculitis / Q000209:etiology / Q000473:pathology* / Q000628:therapy
chemical_list: D000893:Anti-Inflammatory Agents; D013256:Steroids