A steroid-resistant cryptogenic multifocal ulcerous stenosing enteritis.

Area of Science:

• Gastroenterology
• Internal Medicine
• Rare Diseases

Background:

• Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare, chronic, and recurrent gastrointestinal disorder of unknown cause.
• It typically presents with shallow ulcers and submucosal thickening without systemic inflammation.

Observation:

• A young woman diagnosed with CMUSE experienced capsule endoscopy retention requiring surgical intervention.
• Following disease relapse, the patient received both budesonide and methylprednisolone, but neither halted the progression of CMUSE.

Findings:

• The case illustrates a CMUSE patient refractory to standard corticosteroid treatments, including budesonide and methylprednisolone.
• Despite the chronic and recurrent nature of her illness over 24 years, the patient has shown no signs of developing carcinoma.

Implications:

• This case underscores the challenges in managing steroid-resistant CMUSE.
• It suggests a potential need for exploring novel therapeutic approaches for patients with refractory CMUSE.
• Long-term follow-up is crucial for monitoring CMUSE patients, even those with apparent disease stability.