Non-specific multiple ulcers of the small intestine unrelated to non-steroidal anti-inflammatory drugs

Matsumoto, Takayuki; Iida, Mitsuo; Matsui, Toshiyuki; Yao, Takashi; Watanabe, Hideki; Okabe, Hidetoshi

doi:10.1136/jcp.2003.015735

Cited by 71 publications

(52 citation statements)

References 23 publications

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“…Although small intestinal ulcers unrelated to use of NSAIDs or enteric-coated drugs are uncommon, a number of such patients have been reported (1). cPLA 2α deficiency may be considered in the molecular differential diagnosis of such ulcers, particularly those with onset early in life.…”

Section: Discussionmentioning

confidence: 99%

Inherited human cPLA2α deficiency is associated with impaired eicosanoid biosynthesis, small intestinal ulceration, and platelet dysfunction

Adler¹,

Cogan²,

Phillips³

et al. 2008

J. Clin. Invest.

101

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Cytosolic phospholipase A 2α (cPLA 2α ) hydrolyzes arachidonic acid from cellular membrane phospholipids, thereby providing enzymatic substrates for the synthesis of eicosanoids, such as prostaglandins and leukotrienes. Considerable understanding of cPLA 2α function has been derived from investigations of the enzyme and from cPLA 2α -null mice, but knowledge of discrete roles for this enzyme in humans is limited. We investigated a patient hypothesized to have an inherited prostanoid biosynthesis deficiency due to his multiple, complicated small intestinal ulcers despite no use of cyclooxygenase inhibitors. Levels of thromboxane B 2 and 12-hydroxyeicosatetraenoic acid produced by platelets and leukotriene B 4 released from calcium ionophore-activated blood were markedly reduced, indicating defective enzymatic release of the arachidonic acid substrate for the corresponding cyclooxygenase and lipoxygenases. Platelet aggregation and degranulation induced by adenosine diphosphate or collagen were diminished but were normal in response to arachidonic acid. Two heterozygous single base pair mutations and a known SNP were found in the coding regions of the patient's cPLA 2α genes (p.[Ser111Pro]+[Arg485His; Lys651Arg]). The total PLA 2 activity in sonicated platelets was diminished, and the urinary metabolites of prostacyclin, prostaglandin E 2 , prostaglandin D 2 , and thromboxane A 2 were also reduced. These findings characterize what we believe is a novel inherited deficiency of cPLA 2 .

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Section: Discussionmentioning

confidence: 99%

Inherited human cPLA2α deficiency is associated with impaired eicosanoid biosynthesis, small intestinal ulceration, and platelet dysfunction

Adler¹,

Cogan²,

Phillips³

et al. 2008

J. Clin. Invest.

101

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“…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%

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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“…We have previously reported on an unusual form of enteropathy of unknown etiology, referred to as chronic nonspecific multiple ulcers of the small intestine (CNSU), which is characterized by chronic blood and protein loss through persistent small intestinal ulcers [4][5][6]. Although this rare but distinct clinicopathologic condition was initially reported in the Japanese literature by Okabe and Sakimura [7], the term ''nonspecific'' in its nomenclature which refers to the nonspecific histology of the ulcers has occasionally led to the misinterpretation of the disease as any condition with small intestinal ulcers of undetermined etiology.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathologic features of chronic nonspecific multiple ulcers of the small intestine

Esaki

Umeno

Kitazono

et al. 2015

Clin J Gastroenterol

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Non-specific multiple ulcers of the small intestine unrelated to non-steroidal anti-inflammatory drugs

Cited by 71 publications

References 23 publications

Inherited human cPLA2α deficiency is associated with impaired eicosanoid biosynthesis, small intestinal ulceration, and platelet dysfunction

Inherited human cPLA2α deficiency is associated with impaired eicosanoid biosynthesis, small intestinal ulceration, and platelet dysfunction

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

Clinicopathologic features of chronic nonspecific multiple ulcers of the small intestine

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