Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE): a 20-year single-center clinical and radiologic experience

Objective: The purpose of this article is to describe clinical and imaging characteristics of confirmed cases of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE). Methods: Retrospective review of electronic medical records identified patients considered for a diagnosis CMUSE over 20-years in a single large tertiary center. Clinical data were abstracted and diagnosis was confirmed based on published criteria. Two GI radiologists reviewed CT and MR enterography (CTE/MRE) exams in consensus of confirmed patients to characterize the imaging features of CMUSE. Results: Eight patients with confirmed CMUSE diagnosis were included for image review, and 9 CTEs and 1 MRE were analyzed. Most patients were males (75%) with a median age at diagnosis of 59.5 years (25–71) presenting with iron deficiency anemia (75%). Patients were commonly refractory (87.5%) to their first therapy, including steroids, with half being refractory to surgical intervention. Major imaging features included multiple (≥ 5; 88%; 7/8), short (< 2 cm; 100%; 8/8), circumferential (100%; 8/8) strictures with moderate wall thickening (6–9 cm), and stratified hyper enhancement (100%; 8/8) located in the ileum (100%; 8/8). Median proximal small bowel dilation was 2.95 cm (2.5–4.1 cm). No CMUSE cases demonstrated penetrating disease (e.g., abscess, fistula). Conclusion: CT and MR enterography are invaluable tools in the multidisciplinary diagnostic evaluation of CMUSE, a rare cause of small bowel strictures with overlapping clinical and imaging features of Crohn’s disease and NSAID enteropathy.