Background Behçet’s disease (BD) is a rare systemic vasculitis of unknown origin. The treatment of BD aims at reducing inflammatory reactions by using immunomodulatory or immunosuppressive agents. Especially in severe curses of the disease, an off-label therapy with TNF-antagonists may be used.
Objectives While there are some studies with infliximab (IFX), for adalimumab (ADA) only case-reports exist. This is why we aimed at analyzing our cohort for the use and efficacy of ADA in various manifestations of BD.
Methods We retrospectively searched our database for patients with BD at the University Hospital of Tuebingen between 01/2005 and 01/2013. Besides treatment, the individual manifestations, disease activity by using the BD Current Activity Form (BD CAF) and humoral inflammation parameters were assessed. Decrease of BDACF was counted as response to treatment whereas any new manifestation was counted relapse or refractory disease, respectively.
Results Altogether 294 patients have been identified fulfilling the criteria of the international study group of BD. 23 patients (15 male) with a median age of 41 (23-62) years received ADA for a median of 30.0 months (6-72). All patients were treated with 40 mg ADA subcutaneously every second week.
Eight of the 23 patients treated with ADA were switched from IFX. Three of these five patients suffered from recurrent oral or genital aphthosis, two patients had panuveitis, one patient a severe cerebral ischemia, one severe mucocutaneous manifestations and one showed large vessel vasculitis. In 4 of these patients IFX was switched to ADA for better convenience of application. In the other four cases IFX had been ineffective.
The other 15 patients had no previous therapy with a TNF-antagonist. Seven of these 15 patients were switched from interferon-alpha due to severe panuvetis with retinal vasculitis. The remaining eight patients had a previous oral immunosuppressive therapy with ongoing disease activity.
In 18 of 23 patients (78%) an improvement was observed with a significant decrease of the Disease Activity Score by 6.2 points on average within 6 months (p=0,012) and a further decline under continued therapy. The inflammatory markers erythrocyte sedimentation rate and C-reactive protein showed no statistic significant decrease. In all of these patients prednisolone dose could be tapered to ≤10mg/day.
In the remaining 5 cases ADA was not effective and we had to stop medication. In two cases a new flare of uveitis despite ADA was observed, in one case a worsening of spondyloarthritis forced us to switch to IFX. Two patients had a severe and treatment refractory mucocutaneous manifestation and had to be switched to high-dose-glucocorticoids.
Conclusions This report, including 23 BD patients treated with ADA, is the largest being published to date. In 78% of the patients a response to treatment could be achieved. Due to the retrospective character of the data and the lack of a control group its significance is limited. Nevertheless ADA seems to be an effective alternative in treatment-refractory cases of BD, in some cases also after a previous failure of IFX. This goes in accordance to the literature with 18 case reports or – series with altogether 89 (1-19) patients.
Disclosure of Interest: None Declared