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

Abstract

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, ...

Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

“…Strictures and ulcers showed no consistent direction such as the mesenteric border lining. Like previous reports, there were no penetrating disease features, including fissure, fistula formation, or abscess[1,3,7]. Mesenteric lymphadenopathy or mesenteric infiltration was rarely seen and there was no other GI involvement except the small intestine.…”

“…Even radiologic information previously reported was relatively simple descriptions and mostly confined to SBS features[1,4,5,7,8]. …”

“…Perhaps the conflicting responsiveness to steroid use in CMUSE patients is linked closely to different disease stages, i.e ., early stage manifesting as multiple shallow ulcers only and the advanced stage as severe strictures. In addition, some investigators reported significant steroid dependency[1,7]. Hence, further investigation with more collective data is necessary regarding proper CMUSE treatment.…”

Cryptogenic Multifocal Ulcerous Stenosing Enteritis: A Review of the Literature

“…Nowadays, owing to deep enteroscopy, it is possible to investigate the entire small intestine [18–21], take numerous biopsy specimens, and utilise other advanced diagnostic methods like flow cytometry and/or immunohistochemistry. That is why several distinct entities can be newly recognised [3]. …”

“…Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare illness characterised by chronic or relapsing subileus or ileus status resulting from multiple small intestinal fibrous strictures, multiple shallow ulcers of the small bowel, and favourable therapeutic effect of systemic glucocorticosteroids [1–3]. …”