Cryptogenic Multifocal Ulcerating Stenosing Enteropathy(CMUSE) and/or Chronic Non-specific Multiple Ulcers of the Small Intestine(CNSU) and Non-granulomatous Ulcerating Jejunoileitis (NGUJI)
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Purpose of Review
The purpose is to make aware of the existence of the rare and exclusive small intestine (SI) diseases, namely cryptogenic multifocal ulcerating stenosing enteropathy (CMUSE) or chronic non-specific multiple ulcers of the small intestine (CNSU) and non-granulomatous ulcerating jejunoileitis (NGUJI). The article will elucidate their epidemiology, pathogenesis, clinical features, diagnosis, differentiating features and management.
Recent papers have published the clinical features and diagnostic criteria of CMUSE/CNSU and NGUJI. CNSU/CMUSE is caused by gene mutations involved in the prostaglandin pathways. Although capsule endoscopy can detect these lesions, it carries a risk of retention. TNF antagonists and azathioprine have shown response in few cases.
CMUSE/CNSU and NGUJI are uncommon diseases that cause relapsing SI obstruction and bleed due to short-segment strictures and multiple shallow ulcers. This article focuses on current knowledge and novel insights regarding their pathogenesis, genetics, clinical features, diagnostic criteria and management. Multicentric clinical and genetic studies are the need of the hour.
KeywordsChronic stenosing enteropathy Chronic enteropathy associated with the SLCO2A1 gene (CEAS) Neuromuscular and vascular hamartoma (NVHM) Capsule endoscopy
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Conflict of Interest
The author declares that there is no conflict of interest.
Human and Animal Rights and Informed Consent
All reported studies/experiments with human or animal subjects performed by the author have been previously published and complied with all applicable ethical standards.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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