Clinical Characteristics and Treatment Outcomes of Cryptogenic Multifocal Ulcerous Stenosing Enteritis in Korea

• DOI: 10.1007/s10620-015-3595-y
• Publication Date: 2015-02-23T10:20:01Z
• Authors: Sook Hee Chung; Sang Un Park; Jae Hee Cheon; Eun Ran Kim; Jeong-Sik Byeon; Byong Duk Ye; Bora Keum; Ki-Nam Shim; Sung-Ae Jung; Jin-Oh Kim; Seong Ran Jeon; Hyun Joo Song; Jeong Seop Moon; Dong Kyung Chang
• Keywords: Adult; Male; Gastrointestinal; Ileal Diseases/pathology; 610; Constriction, Pathologic; Disease-Free Survival; Endoscopy, Gastrointestinal; Jejunal Diseases/etiology*; 03 medical and health sciences; Enteritis/therapy; 0302 clinical medicine; Recurrence; Ulcer/therapy; Diagnosis; 616; Republic of Korea; Ulcer/etiology*; Humans; Treatment outcome; Pathologic/surgery; Ulcer; Pathologic/pathology; Retrospective Studies; Cryptogenic multifocal ulcerous stenosing enteritis; Ileal Diseases; Ileal Diseases/therapy; Endoscopy; Jejunal Diseases; Middle Aged; Enteritis/complications*; Jejunal Diseases/therapy; Constriction; Ulcer/pathology; Ileal Diseases/etiology*; Enteritis; Abdominal Pain; 3. Good health; Abdominal Pain/etiology; Characteristics; Jejunal Diseases/pathology; Female; Pathologic/etiology; Enteritis/diagnosis

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease that is characterized by multiple, recurring small intestinal ulcers with stenosis of unknown causes. The aim of this study was to investigate the clinical characteristics and the treatment outcomes of patients with CMUSE in Korea.We performed a multicenter study to retrospectively analyze clinical data from 20 patients who suffered from CMUSE between 1984 and 2012. Their clinical characteristics and long-term disease courses were investigated.The most common initial symptom of CMUSE was abdominal pain (14/20, 70 %). Small bowel series (13/20, 65 %), double-balloon enteroscopy (12/20, 60 %), CT enterography (12/20, 60 %), and capsule endoscopy (10/20, 50 %) were used to diagnose CMUSE. The strictures of the patients were located in the jejunum (5/20, 25 %), ileum (7/20, 35 %), and both jejunum and ileum (6/20, 30 %). The number of patients in a state of remission, persistent disease, and relapse at the end of follow-up were 13/20 (65 %), 2/20 (10 %), and 5/20 (25 %), respectively. The median relapse-free survival was of 67.1 months. Seventy-five percent relapse-free survivals for female and male patients were 93 and 9 months, respectively (P = 0.031).CMUSE is difficult to diagnose and is an easily relapsing disease. Female patients might have a better prognosis than male patients in terms of the relapse-free time.