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

Perlemuter, Gabriel; Guillevin, Loı̈c; Legman, P.; Weiss, Lindsay; Couturier, Daniel; Chaussade, Stanislas

doi:10.1136/gut.48.3.333

Cited by 85 publications

(109 citation statements)

References 19 publications

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“…We do not agree with Perlemuter et al (37,38) that CMUSE could be termed as a type of "atypical vasculitis". Any type of vasculitis of any cause, if found, should be assigned simply as "vasculitis" not CMUSE.…”

Section: Discussioncontrasting

confidence: 43%

“…Most of these patients become corticodependent (37,44). All our three patients positively responded to this treatment and indeed 2 of 3 became dependent on glucocorticosteroids.…”

Section: Discussionmentioning

confidence: 88%

“…Perlemuter et al (37) reported 12 cases of CMUSE hospitalised between 1965 and 1993. This was a retrospective analysis of medical records based on queries sent to 220 French gastroenterology departments.…”

Section: Discussionmentioning

confidence: 99%

“…Only about fifty cases of CMUSE have been published so far (9,33,34,37,46,42) but this entity has probably been considerably underdiagnosed or misdiagnosed, mostly with Crohn's disease and non-steroidal anti-inflammatory drugs (NSAIDs) induced enteropathy. Perlemuter et al (37) reported 12 cases of CMUSE hospitalised between 1965 and 1993.…”

Section: Discussionmentioning

confidence: 99%

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Severe Cryptogenic Multifocal Ulcerous Stenosing Enteritis. A Report of Three Cases and Review of the Literature

Kohoutová

Bureš

Tyčová

et al. 2010

Acta Med. (Hradec Kralove, Czech Repub.)

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IntroductionCryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare condition characterised by chronic or relapsing moderate ileous episodes resulting from multiple small intestinal strictures, multiple shallow ulcers of the small bowel and beneficial therapeutical effect of glucocorticosteroids (1, 2). Japanese gastroenterologists call this syndrome chronic non-specific multiple ulcers of the small intestine (CNSU) (3). The aetiology of CMUSE has not been clarified yet and pathogenesis is still poorly understood. Some authors even doubt the real existence of this entity.Patients with CMUSE are often referred for surgery because of symptomatic small intestinal strictures and several resections of the small bowel had to be performed.We report three cases of CMUSE diagnosed within the past 10 years at a single tertiary centre, where more than eight thousand GI endoscopies are performed per year. Review of available relevant literature is provided. Case reports Case 1A 35-year-old woman was admitted to our department because of chronic diarrhoea, colicky abdominal pain, weight loss (16 kg during previous five years) and repeated moderate ileous episodes. She was cachectic (body-mass index 12.8 kg/m 2 ). Laboratory tests showed iron-deficiency anaemia (haemoglobin 96 g/L), thrombocytosis (459.10 9 g/L) and proteino-energy malnutrition (serum prealbumin 0.17 g/L). Abdominal ultrasound revealed strenuous small intestinal peristalsis and a large volume of fluid in the intestinal loops. Enteroclysis provided no further information. Gastroscopy and colonoscopy were normal. Push-enteroscopy found multiple shallow ulcers of the distal duodenum and proximal jejunum ( Fig. 1) and increased stagnant fluid content in the proximal part of the small bowel. A non-specific inflammatory process was detected at histology and electron microscopy of small intestinal biopsy specimens. Coeliac disease, lymphoma, Crohn's disease and vasculitis were excluded. No infective agent was found. Total parenteral nutrition and glucocorticosteroids improved her general condition including nutritional status (serum albumin 35.8 g/L, prealbumin 0.29 g/L, body-mass index 13.4 kg/m 2 ). The patient was discharged on enteral nutrition, glucocorticosteroids (prednisone was gradually replaced by budesonide) and 5-aminosalicylates. She was followed-up at regular controls. Nine months later the patient had to be admitted because of a worsened general condition (frequent watery stools, fatigue, oedemas of lower extremities, fluidothorax and ascites). Laboratory tests revealed hypo-25 CASE REPORT Summary: Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare condition characterised by chronic or relapsing moderate ileous episodes resulting from multiple small intestinal strictures, multiple shallow ulcers of the small bowel and favourable therapeutical effect of glucocorticosteroids. The aim of this paper was to evaluate three cases of CMUSE diagnosed within 10 years at a tertiary gastroenterology centre. Three females (35, 50, ...

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Section: Discussioncontrasting

confidence: 43%

“…Most of these patients become corticodependent (37,44). All our three patients positively responded to this treatment and indeed 2 of 3 became dependent on glucocorticosteroids.…”

Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Severe Cryptogenic Multifocal Ulcerous Stenosing Enteritis. A Report of Three Cases and Review of the Literature

Kohoutová

Bureš

Tyčová

et al. 2010

Acta Med. (Hradec Kralove, Czech Repub.)

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“…Cryptogenic multifocal ulcerous stenosing enteritis is a rare idiopathic cause of ulceration and stricture of small bowel, which can be presented with acute or chronic GI bleeding, symptoms of bowel obstruction, abdominal pain, anemia, hypoalbuminemia, and perforation (1)(2)(3)(4)(5).…”

Section: Discussionmentioning

confidence: 99%