Abdominal pain and bloody diarrhea in a 32-year-old woman
TREATING INTESTINAL BEHÇET DISEASE
3. Which is not considered a standard treatment for intestinal Behçet disease?
- Mesalamine (5-ASA)
- Corticosteroids
- Immunosuppressants
- Mycophenolate mofetil
- Surgery
Overall, data on the management of intestinal Behçet disease are limited. The data that do exist have shown that 5-ASA, corticosteroids, immunosuppressants, and surgery are options, but not mycophenolate mofetil.
Consensus recommendations from the Japanese IBD Research Group,24 published in 2007, included 5-ASA, corticosteroids, immunosuppressants, enteral and total parenteral nutrition, and surgical resection. In 2014, the group published a second consensus statement, adding anti-tumor necrosis factor (TNF) agents as standard therapy for this disease.22
Mycophenolate mofetil has not been shown to be effective in the treatment of mucocutaneous Behçet disease,25 although it may be effective in the treatment of its neurologic manifestations.26 Data regarding its efficacy in intestinal Behçet disease are sparse.
Differences in treatment for Crohn and Behçet disease
Although the treatment options are comparable for Behçet disease and Crohn disease, certain features differ.
Doses of 5-ASA and immunnosuppressive agents are typically higher in Crohn disease. For example, the optimal dose of 5-ASA is up to 3 g/day for Behçet disease but up to 4.8 g/day for Crohn disease.
Standard dosing for azathioprine is 50 to 100 mg/day for Behçet disease but 2 to 2.5 mg/kg/day (eg, 168 to 210 mg/day for a 185-lb patient) for Crohn disease.
In addition, evidence supporting the use of biologic agents such as anti-TNF agents or vedolizumab is more abundant in Crohn disease.
Finally, data on monitoring drug levels of immunomodulators or biologics are available only for patients with Crohn disease, not Behçet disease. Thus, an accurate diagnosis is important.
CASE CONTINUED: EMERGENCY LAPAROTOMY
Our patient continued to experience abdominal pain and bloody diarrhea despite receiving corticosteroids intravenously in high doses. We were also considering anti-TNF therapy.
At this point, CT of her abdomen and pelvis was repeated and showed free intraperitoneal air consistent with a perforation of the transverse colon.
She underwent emergency exploratory laparotomy. Intraoperative findings included pneumoperitoneum but no gross peritoneal contamination, extensive colitis with a contained splenic flexure perforation, and normal small-bowel features without evidence of enteritis. Subtotal colectomy, implantation of the rectal stump into the subcutaneous tissue, and end-ileostomy were performed.
After 23 days of recovery in the hospital, she was discharged on oral antibiotics and 4 weeks of steroid taper.
PROGNOSIS OF INTESTINAL BEHÇET DISEASE
4. What can the patient expect from her intestinal Behçet disease in the future?
- The disease is cured after resection of the diseased segments
- Behçet disease is a progressive lifelong disorder that can recur after surgery
Like Crohn disease, Behçet disease should be considered a lifelong progressive disorder, even after surgical resection of diseased segments.
It is unclear which patients will have a complicated disease course and need treatment with stronger immunosuppression. In patients with intestinal Behçet disease whose disease is in remission on thiopurine therapy, the 1-year relapse rate has been reported as 5.8%, and the 5-year relapse rate 51.7%.27,28 After surgical resection, the 5-year recurrence rate was 47.2%, and 30.6% of patients needed repeat surgery.29 Predictors of poor prognosis were younger age, higher erythrocyte sedimentation rate, higher C-reactive protein level, low albumin level at diagnosis, and a high disease-activity index for intestinal Behçet disease.30
The Korean IBD Study Group has developed and validated a disease activity index for intestinal Behçet disease.28 The index has a list of weighted scores for 8 symptoms, which provides for a more objective assessment of disease activity for determining the best treatment approach.
CASE CONTINUED
The patient has continued with her follow-up care and appointments in gastroenterology, rheumatology, and dermatology clinics. She still complains of intermittent abdominal pain, occasional bleeding at the rectal stump, intermittent skin lesions mainly in the form of pustular lesions, and intermittent joint pain. If symptoms persist, anti-TNF therapy is an option.
