Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

Abstract

AIMTo investigate the characteristic radiologic findings of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) which can be differentiated from other similar bowel disease and to assess their clinical behavior.METHODSTwenty pathologically and clinically confirmed CMUSE patients (males:females = 8:12; mean age: 40.4 years) between March 2002 and August 2015 from seven academic centers in South Korea were retrospectively reviewed. We evaluated small bowel series (SBS; n = 25), computed tomography (CT) enterography (n = 21), magnetic resonance (MR) enterography (n = 2), and abdominopelvic CT (n = 18) images, focusing on enteric and perienteric manifestations. Any change in radiologic features during follow-up period was recorded. We evaluated clinical data including presenting symptoms, laboratory finding and presence of relapse from electronic medical records. Histopathologic findings were also evaluated.RESULTSThe main symptoms were abdominal pain (n = 12) and anemia (n = 10). All patients showed small bowel strictures (n = 52, mean: 2.6 per patient) on initial CT/MR, located in the ileum (n = 47) or jejunum (n = 5). Strictures showed short-length (mean: 10.44 mm) and circumferential bowel wall thickening (mean: 5.56 mm) with layered enhancement (n = 48) that were also noted on initial SBS (n = 36) with shallow ulcers (n = 10). Some ulcerative lesions or wall thickening progressed into strictures on follow-up SBS/CT, and some strictures revealed recurrent ulceration on follow-up SBS. There were no penetrating disease features like fistula or abscess and no gastrointestinal tract involvement except the small bowel. Nine patients experienced disease recurrence (median relapse-free period: 32 mo) even post-operatively. Histopathologic features of surgically resected specimens were characterized as multiple superficial ulcerations confined to mucosa or submucosa and multiple strictures.CONCLUSIONUnder characteristic radiologic findings with multiple short-segmental strictures and/or shallow ulcers of the small intestine, CMUSE should be considered when assessing patients with recurrent abdominal pain and anemia.

A male Korean who was diagnosed with chronic enteropathy associated withSLCO2A1(CEAS): case report with literature review

“…Moreover, we believe that there might have been cases of CEAS misdiagnosed as CMUSE or other disorders, especially in Asian countries like Korea and China. 8 …”

Retrieval of retained capsule endoscopy by device‐assisted enteroscopy in patients with cryptogenic multifocal ulcerous stenosing enteritis

“…7 CTE or magnetic resonance enterography can be used in the diagnosis of CMUSE, showing an increased enhancement of intestinal mucosa, short segmental stenoses and a slightly thickened intestinal wall; however, short stenoses can easily be missed. 8 The small intestinal ulcers in CMUSE are shallow and flat.…”

“…At present, intestinal stenoses can be treated with DAE. 8 Several studies have demonstrated that endoscopic balloon dilation (EBD) using DBE for small intestinal strictures is effective and safe. 7 Based on our findings, EBD may be considered as a first-line treatment option for CD patients with small intestinal stenoses to reduce the need for surgical intervention over the patient's lifetime, unless F I G U R E 2 In Case 2, A, A capsule endoscopy showed scarred ulcers and stenoses in the small intestine.…”

NSAID Enteropathy: A Review of the Disease Entity and Its Distinction from Crohn’s Enteropathy

“…45,46 Multifocal stenosing ulcerations are known in Crohn's, vasculitis, radiation enteritis, and cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) among other conditions. 47 CMUSE is defined as unexplained small intestinal multiple strictures and ulcerations of unknown origin and is thought to be an atypical form of vasculitis. It is seen in middle-aged patients with no evidence of systemic inflammation.…”