Cryptogenic, Multifocal, Ulcerous, and Stenosing Enteritis as a Manifestation of Enterocolic Venopathy

Hussey, Séamus; Bourke, Billy; Broderick, Annemarie; Phelan, Eimear; McDermott, Michael

doi:10.1097/mpg.0b013e3181661ad1

Cited by 8 publications

(3 citation statements)

References 16 publications

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“…Recently, Fraile et al (16) reported an association of CMUSE with X-linked recessive reticulate pigmentary disorder. Hussey et al (23) published a case of CMUSE as a manifestation of enterocolic venopathy.…”

Section: Discussionmentioning

confidence: 99%

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: Discussionmentioning

confidence: 99%

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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“…CMUSE was also reported in association with homozygous deletion mutations in cytosolic phospholipase A2- α [41]. Hussey et al [42] published a case of CMUSE as a manifestation of enterocolic venopathy. …”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Cryptogenic Multifocal Ulcerous Stenosing Enteritis: A Review of the Literature

Kohoutová

Bártová

Tachecí

et al. 2013

Gastroenterology Research and Practice

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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.

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“…3 Complement deficiency has also been attributed to CMUSE in the context of a vasculitic process. 4,5 In our patient, there was evidence of venopathy, but testing including complement levels, markers of autoimmunity, imaging evaluations, and the lack of additional systemic manifestations did not suggest a vasculitic process.…”

Section: Discussionmentioning

confidence: 58%

Cryptogenic Multifocal Ulcerative Sclerosing Enteritis: A Curious Case of Intestinal Obstruction in the Setting of Human Immunodeficiency Virus

et al. 2019

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Cryptogenic multifocal ulcerative sclerosing enteritis (CMUSE) is a rare clinical entity characterized by chronic, relapsing episodes of ileus and obstruction resulting from superficial ulcerating lesions of the small intestine with a clinical course that responds favorably to corticosteroids. We report a case of CMUSE arising in a patient with a history of human immunodeficiency virus infection. This case highlights the unique pathology of CMUSE as well as the potential pathogenesis of this atypical clinical entity.

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Cryptogenic, Multifocal, Ulcerous, and Stenosing Enteritis as a Manifestation of Enterocolic Venopathy

Cited by 8 publications

References 16 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

Cryptogenic Multifocal Ulcerative Sclerosing Enteritis: A Curious Case of Intestinal Obstruction in the Setting of Human Immunodeficiency Virus

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