Cryptogenic multifocal ulcerous stenosing enteritis

Spencer, Hal; Kitsanta, Panagiota; Riley, Stuart

doi:10.1258/jrsm.97.11.538

Cited by 16 publications

(10 citation statements)

References 2 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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: 50%

See 1 more Smart Citation

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.)

View full text Add to dashboard Cite

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, ...

show abstract

“…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: 50%

“…Matsumoto (33) did not reveal any arteritis in his series as well. Perlemuter et al (38) suggested the association of CMUSE with C2 complement deficiency; however, this was not confirmed by other authors (44). Recently, Fraile et al (16) reported an association of CMUSE with X-linked recessive reticulate pigmentary disorder.…”

Section: Discussionmentioning

confidence: 95%

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.)

View full text Add to dashboard Cite

show abstract

“…CMUSE is an extremely rare illness. Only about sixty cases of CMUSE have been published so far [ 1 – 17 , 22 – 24 ], but this entity has probably been considerably underdiagnosed or misdiagnosed, mostly with Crohn's disease and nonsteroidal anti-inflammatory drugs- (NSAIDs)-induced enteropathy [ 3 ]. Perlemuter et al [ 1 ] reported 12 cases of CMUSE hospitalised in France between 1965 and 1993.…”

Section: Epidemiologymentioning

confidence: 99%

Cryptogenic Multifocal Ulcerous Stenosing Enteritis: A Review of the Literature

Kohoutová

Bártová

Tachecí

et al. 2013

Gastroenterology Research and Practice

View full text Add to dashboard Cite

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.

show abstract

“…A syndrome that is characterized by intermittent episodes of small-intestinal obstr uction caused by benign multifocal ulcerated stenosis has been described, largely, but not entirely, in the French literature [1][2][3][4][5][6][7][8][9][10] . This disorder has also been termed "cryptogenic multifocal ulcerous stenosing enteritis" (CMUSE) and has been reported to localize largely within the jejunum or proximal ileum.…”

Section: Introductionmentioning

confidence: 99%

Multifocal stenosing ulceration of the small intestine

Freeman¹

2009

WJG

View full text Add to dashboard Cite

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.

show abstract

Cryptogenic multifocal ulcerous stenosing enteritis

Cited by 16 publications

References 2 publications

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

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

Cryptogenic Multifocal Ulcerous Stenosing Enteritis: A Review of the Literature

Multifocal stenosing ulceration of the small intestine

Contact Info

Product

Resources

About