What is the treatment for intestinal Behcet's disease?

Treatment for Intestinal Behçet's Disease

For intestinal Behçet's disease, agents such as sulfasalazine, corticosteroids, azathioprine, TNF-α antagonists, and thalidomide should be tried first before surgery, except in emergencies. 1

Disease Characteristics and Initial Approach

Intestinal Behçet's disease is characterized by:

• Single or multiple deep penetrating ulcers, primarily in the terminal ileum, ileocecal region, and colon
• Tendency for perforation, which may require emergency surgical intervention
• Fluctuating disease course with episodes of relapse and remission

Treatment Algorithm

First-line Treatments:

1. 5-Aminosalicylic acid (5-ASA) derivatives

   • For milder gastrointestinal involvement
   • Often used as initial therapy

2. Corticosteroids

   • For acute exacerbations to promote rapid healing of ulcers
   • Typical regimen: High-dose glucocorticoids during flares
   • Caution: Potential concern about facilitating perforation in high-risk patients, though no definitive data confirms this 1

3. Azathioprine (2.5 mg/kg/day)

   • For more severe cases
   • Has been shown to decrease re-operation rates in patients requiring surgery 1
   • Should be considered as maintenance therapy after surgery

Second-line/Refractory Disease Treatments:

1. TNF-α antagonists (infliximab, adalimumab)

   • For severe and/or refractory disease 1
   • Emerging evidence shows effectiveness in treating intestinal Behçet's disease 2

2. Thalidomide

   • For resistant and complicated cases
   • Case reports support its use 1

Surgical Management:

• Urgent surgical consultation is necessary in cases of:
  • Perforation
  • Major bleeding
  • Intestinal obstruction 1
• Surgical procedures may include ileocolectomy or hemicolectomy
• High recurrence and re-operation rates at long term
• Medical treatment with immunosuppressives should be tried first except in emergencies 1

Poor Prognostic Factors

Patients with the following characteristics may require more aggressive treatment:

• Young age at diagnosis
• Higher disease activity at diagnosis
• Volcano-type ulcers
• Absence of mucosal healing
• Elevated C-reactive protein levels
• Prior history of surgery
• Lack of initial response to medical therapy 3

Treatment Monitoring and Adjustments

• Regular assessment of clinical symptoms and endoscopic findings
• Monitor for complications such as perforation, bleeding, and obstruction
• Adjust therapy based on disease activity and treatment response
• Consider maintenance therapy after achieving remission to prevent recurrence

Important Considerations

• There are no controlled trials specifically for intestinal Behçet's disease treatment
• Evidence is largely based on retrospective observational data
• Avoid cyclosporine A in patients with CNS involvement due to potential neurotoxicity 1
• Anticoagulants are not recommended as primary treatment as the pathology is inflammation of vessel walls rather than thrombophilia 4

The management of intestinal Behçet's disease remains challenging due to the rarity of the disease and lack of controlled studies, but early aggressive treatment with immunosuppressive agents is crucial to prevent serious complications and improve outcomes.