BACKGROUND: Introduction of anti-TNF treatment has greatly improved prognosis of Behcet's disease (BD). Withdrawal of anti-TNF treatment in chronic inflammatory arthritis or bowel disease has been associated with sustained remission in subsets of patients. Herein, we examined the probability of very long-term quiescence after withdrawal of TNF inhibitors in BD. METHODS: Retrospective longitudinal outcome single-center study focusing on BD patients who discontinued successful ant-TNF treatment since 2001. Endpoint was their proportion with sustained clinical remission for 5 years after withdrawal. RESULTS: Thirty-three patients with severe BD refractory to non-biologic treatment (mean age 47.5 ± 11.5 years, 55% men) achieved TNF inhibitor-induced remission for a median of 2 years (IQR [1-2.6]). TNF inhibitors had been given for sight-threatening disease (28/33, 82%), for mucocutaneous (3/33), central nervous system (2/33) and gastrointestinal involvement (1/33). After withdrawal, BD remained in remission in 15/33 patients for 6.6 to 20.6 years (mean 13.5 ± 3.8). Conversely, 18/33 patients relapsed after a median of 9.5 months [IQR 8-12, range 4-32] following withdrawal but retreatment with TNF inhibitors was effective in 13/18. Of them, 9/13 discontinued for a second time and achieved again the study's endpoint, remaining in remission for median of 7.4 years ([IQR 5-9.3, range 5-15 years). Study's end-point was met by 24/33 patients (73%)
17and 7 patients remain any-drug free or on azathioprine only, respectively. CONCLUSION: Discontinuation of successful anti-TNF treatment is frequently associated with durable very long-term remission in severe BD. Additional studies are needed since relapses not responding to anti-TNF re-treatment may occur.