CRYPTOGENIC MULTIFOCAL ULCEROUS STENOSING ENTERITIS (CMUSE) IN THE ENTEROSCOPIC ERA

Background and study aims:

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare and overlooked small bowel disorder of uncertain origin. Differential diagnosis from Crohn's disease, NSAID-associated enteritis and enteric lymphoma is challenging.

We report the enteroscopic (capsule and device assisted) diagnostic process and management of a series of patients with CMUSE.

Patients and methods:

From November 2016 to November 2018, in all patients presenting a CMUSE-like endoscopic picture we have prospectively recorded: clinical data, enteroscopic, histologic and radiologic findings.

Results:

Among 561 enteroscopies (277 DBEs, 284 VCE) performed in 417 consecutive patients, the final diagnosis of CMUSE was made in 6 (5 males, median age 76 years, range 25 – 83 years) resulting in a prevalence of 1.5%. Indication to enteroscopy was iron deficiency anemia (IDA) (Hb 10.9 ± 2.7 g/dl). At baseline, only one patient assumed NSAIDs (cardioaspirin). All patients underwent DBE and cross-sectional imaging (EnteroMR or EnteroCT or Small bowel-US). VCE was performed as first examination in 4 cases with one case of capsule retention (DBE retrieved). No transmural nor mesenteric involvement was reported by cross-sectional imaging. The DBE showed isolated small intestinal ulcers and stenotic lesions, confirming the findings of the previous investigations. The surrounding mucosa appeared normal. Five patients had an isolated lesion and 1 had multiple ones. In 5 patients stenosis were localized in the ileum, in 1 case in the jejunum. Histopathological reports were nonspecific (chronic flogosis). Over a mean follow-up of 18 months, systemic corticosteroids were the first-line treatment and 4 were referred to laparoscopic resective surgery.

Conclusion:

Our findings suggest that CMUSE is more frequent than previously reported. Stenotic ulcers are mostly isolated and located in the ileum. DBE and histopathology are pivotal to assess differential diagnosis.