Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency

Perlemuter, Gabriel; Chaussade, Stanislas; Soubrane, Olivier; Degoy, A; Louvel, A; Barbet, P.; Legman, P.; Kahan, André; Weiss, Lindsay; Couturier, Daniel

doi:10.1053/gast.1996.v110.pm8613071

Cited by 52 publications

(53 citation statements)

References 5 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: 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: 99%

“…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: 94%

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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“…The unusual clinical feature was the ataxia due to cerebellar atrophy. The diagnosis of systemic sclerosis was supported by the finding of anticentromere antibodies (associated with the development of non-fibrotic pulmonary hypertension in that condition 3 ) and antinuclear antibodies (present in more than 70% of patients 3,4 ); the anti-SSA antibodies were also consistent with the diagnosis. 3,5 Progressive subacute cerebellar degeneration can be part of a paraneoplastic syndrome, but there was no evidence of neoplasia on investigation or subsequent follow-up.…”

mentioning

confidence: 87%

“…COMMENT CMUSE, first described in 1964 by Deray et al, 3 is an unusual condition, with fewer than twenty cases reported. 4 Most cases present with repeated bouts of small-intestine obstruction. Multiple strictures (2-25) are often present, with frequent relapse following resection.…”

Section: Case Historymentioning

confidence: 99%

Cryptogenic multifocal ulcerous stenosing enteritis

Spencer

Kitsanta

Riley

2004

JRSM

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“…In this issue of Digestive Diseases and Sciences [1], Chung and colleagues from South Korea describe a multicenter case series of ''cryptogenic multifocal ulcerous stenosing enteritis'' or CMUSE, defined using modern endoscopic methods for small intestinal mucosal evaluation and followed up for an extended period. Through these observations, the authors provide novel information regarding the long-term natural history of CMUSE, a specific syndrome initially described more than a half-century ago largely, although not entirely, in the French literature [2][3][4][5][6]. In these early reports, CMUSE was typically associated with multiple shallow mucosal ulcerations, usually in the jejunum or proximal ileum.…”

Section: Background and Significancementioning

confidence: 97%

Small Intestinal Multifocal Stenosing Ulceration

Freeman

2015

Dig Dis Sci

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Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency

Cited by 52 publications

References 5 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

Small Intestinal Multifocal Stenosing Ulceration

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