Gastrointestinal Mucosal Findings in Behçet Syndrome
The gastrointestinal involvement in Behçet syndrome is primarily characterized by deep penetrating ulcers, most commonly located in the terminal ileum, ileocecal region, and colon, which have a tendency to perforate and cause significant morbidity and mortality. 1, 2
Typical Endoscopic Findings
Location of Lesions
- Primary locations:
- Terminal ileum (most common)
- Ileocecal region
- Colon
- Any segment of the gastrointestinal tract can be involved, though less commonly 3, 4
Morphological Characteristics
- Single or multiple deep penetrating ulcers with:
- Punched-out appearance
- Undermining edges
- Well-defined margins 5
- Ulcers tend to be:
- Deep
- Discrete
- Round or oval in shape
- Larger than aphthous ulcers seen in inflammatory bowel disease 1
Atypical Presentations
- In approximately 10% of cases, diffuse ulcerative lesions may be found in the transverse and distal colon, resembling inflammatory bowel disease 5
- These diffuse lesions can sometimes make differentiation from ulcerative colitis or Crohn's disease challenging 6, 7
Clinical Implications of GI Lesions
Complications
- High risk of perforation due to the deep penetrating nature of ulcers 1
- Potential for major bleeding
- Intestinal obstruction
- Fistula formation 2
Diagnostic Considerations
- Gastrointestinal involvement should be confirmed by endoscopy and/or imaging 1
- Important to rule out:
- NSAID-induced ulcers
- Inflammatory bowel disease
- Infections (particularly tuberculosis) 1
Management Approach for GI Involvement
Initial Management
- Medical treatment should be attempted before considering surgery (except in emergencies) 1
- For acute exacerbations: glucocorticoids combined with disease-modifying agents such as 5-ASA or azathioprine 1
Severe/Refractory Cases
- TNF-alpha inhibitors (particularly infliximab) or thalidomide should be considered 1
- Urgent surgical consultation is necessary in cases of:
- Perforation
- Major bleeding
- Obstruction 1
Surgical Considerations
- High recurrence and re-operation rates after surgical procedures 1
- Maintenance therapy with azathioprine may decrease re-operation rates in patients requiring surgery 1
Monitoring
- Regular endoscopic follow-up is important to assess mucosal healing and treatment response
- Careful monitoring for complications is essential given the high risk of perforation and bleeding 1
The deep, penetrating nature of ulcers in Behçet syndrome distinguishes it from other inflammatory bowel diseases and represents a significant risk for serious complications. Early recognition of these characteristic lesions is crucial for appropriate management and prevention of morbidity and mortality.