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P414. Management of gastrointestinal Behçet's disease refractory to conventional treatment

I. Hatemi1, G. Hatemi2, Y. Erzin1, A.F. Celik1, 1Istanbul University Cerrahpasa Medical Faculty, Gastroenterology, Istanbul, Turkey, 2Istanbul University Cerrahpasa Medical Faculty, Rheumatology, Istanbul, Turkey

Background

Based on non-placebo controlled studies, the major medical therapy options of gastrointestinal involvement of Behçet's disease (GIBD) are corticosteroids (CS) and 5-ASA compounds for mild to moderate, azathioprine (AZA) for moderate to severe cases. There are limited number of medications for induction and maintaining remission. In this study we summarize our experience with refractory GIBD cases.

Methods

We retrospectively reviewed the charts of all GIBD patients who are defined as refractory to conventional medical therapies. Chart records of treatment responses, endoscopic, and inpatients outpatients files were considered.

Results

The total number of GIBD patients and refractory GIBD patients are 53 and 13 (25% of all GIBS patients) respectively. GIBD patients is 3.5% of our IBD outpatient clinic. We use either anti-TNF alpha blockers or thalidomide for medical management of refractory GIBD patients. For 4 refractory cases of GIBD, thalidomide and for another 9 cases anti-TNF were used (adalimumab in 2 cases, infliximab in 6 cases and etanercept in 1 case, both of the adalimumab cases were also unresponsive to infliximab). In ten out of 13 cases we have seen clinical response (76.9%). Three patients remain unresponsive and one of them died of infectious complications. In one patient remission was obtained by surgery and in 1 case with myelodysplastic syndrome by hemapoetic stem cell transplantation. Nine of these 13 patients were using concomitantly AZA and resting 4 patients have had AZA. Endoscopic remission was 70% (8 of 12) in the whole group.

Table: Treatment of refractory cases
No.LocationPrevious treatmentTreatment for refractory diseaseConcomitant therapyOutcomeDuration of remission
1CAZA/CS/INFADANR
2ICSAL/AZA/CS/INF/BUDADACS AZA BUDCR: 2W
ER: 12W
LR: 2W
15M
3ICAZA/CS/CSA/THDINFAZACR: 1W
ER: 8W
LR: 2W
50M
4TI5ASA/CS/AZAINFAZACR: 2W
ER: 24W
LR: 1W
72M
5CAZAINFAZACR: NA
ER: 8W
LR: NA
48M
6ICAZA/CSA/IFN/CSINFCSNR
7C5ASA/AZAINFAZACR: 4W
ER: NA
LR: 2W
6M
8ICCS/AZA/CSA/IFN/INF/ADA/Anakinra/CanakinumabINFAZA/THDCR: 1W
ER: 12W
LR: 1.5W
12M
9ICAZA/CYCEtanerceptCR: 1W
ER: 4W
LR: NA
120M
10ICCS/AZATHDAZACR: 4W
ER: 24W
LR: 4W
72M
11ICAZATHDAZACR: NA
ER: 8W
LR: NA
60M
12DAZATHDNR132M
13CAZATHDAZACR: NA
ER: 8W
LR: NA
144M
ADA, Adalimumab; AZA, azathioprine; BUD, Budesonide; C, Colonic; CR, Clinical remission; CS, corticosteroid; CSA, cyclosporine; D, Duodenum; ER, Endoscopic remission; IC, Ileocolic; INF, Infliximab; IFN, Interferon; LR, Laboratory remission; M, Month; NR, Non response; SAL, Salozopyrine; THD, Thalidomide; TI, Terminal ileum; W, week.

Conclusion

Nearly 25% of GIBD cases are refractory to immunmodulator and CS therapy. The major options in these cases are THD and anti-TNF blockers. With these medications remission can be obtained in approximately 75% of the cases. Stopping the medical treatment even in patients under remission, has no clear cut rules. However, one should remember that prominent vasculitic characteristic of the Behçet's disease and GIBD may have a long term endoscopic remission without new flare.