Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

AIM To 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. METHODS Twenty 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. RESULTS The 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. CONCLUSION Under 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.