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Cryptogenic Multifocal Ulcerating Stenosing Enteropathy(CMUSE) and/or Chronic Non-specific Multiple Ulcers of the Small Intestine(CNSU) and Non-granulomatous Ulcerating Jejunoileitis (NGUJI)

  • Small Intestine (D Sachar, Section Editor)
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Abstract

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 Findings

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.

Summary

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.

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Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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  1. Department of Gastroenterology and Hepatobiliary Sciences, IMS and SUM Hospital, Siksha ‘O’ Anusandhan, deemed to be University, Kalinga Nagar, Bhubaneswar, Orissa, 751003, India

    Ayaskanta Singh

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Singh, A. Cryptogenic Multifocal Ulcerating Stenosing Enteropathy(CMUSE) and/or Chronic Non-specific Multiple Ulcers of the Small Intestine(CNSU) and Non-granulomatous Ulcerating Jejunoileitis (NGUJI). Curr Gastroenterol Rep 21, 53 (2019). https://doi.org/10.1007/s11894-019-0721-6

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