gms | German Medical Science

Background: Neuro-Behçet`s disease (NBD) is categorised into parenchymal or nonparenchymal involvement. For parenchymal CNS involvement corticosteroids, IFNα, azathioprine, cyclophosphamide, methotrexate and TNFα-antagonists are recommended. There is a broad agreement to start therapy giving high pulsed corticosteroids followed by cyclophosphamide. Up to now, the establishment of a maintenance immunosuppressive therapy is not resolved. Azathioprine is the preferred regime. IFNα and TNFα-antagonists have been used successfully in resistant cases. We report three cases with leflunomide as maintenance therapy.

Methods: The activity of Behçet`s disease (BD) was calculated using the BD Current Activity Form (BDCAF) and the Birmingham Vasulitis Activity Score (BVAS). All patients were also monitored by serological markers and Magnetic resonance imaging (MRI) of the brain.

Results: Two patients with BD had a new onset neurological involvement while taking azathioprine. The first one with parenchymal involvement was treated with pulsed corticosteroids and i.v. cyclophosphamide. Because of persistent activity cyclophosphamide was discontinued and infliximab started. The second patient with pseudotumor cerebri and sixth cranial nerve palsy received infliximab as first-line treatment because of a planning pregnancy. A third patient presented with cephalgia, emesis and inflammatory cells in the cerebrospinal fluid received azathioprine after pulsed corticosteroid treatment. Azathioprine had to be stopped because of adverse effects. We decided for an oral maintenance therapy with leflunomide (starting with 15 mg per day) because of inefficacy respectively intolerance. In two cases we achieved a prolongation of the infliximab application intervals up two eight weeks without any neurological flare. The third patient is also nearly asymptomatic under leflunomide and low-dose-prednisolon. All three patients in the further course had a low disease activity (BVAS persistent score: 0-2 , BVAS new / worse score: 0-2, BDCAF: 0-2). The reported symptoms were limited to oral aphthosis, papulopustulosis and arthralgia. Follow up MRI of the brain could not show any signs of activity in all patients.

Conclusion: This is the first report on leflunomide as a maintenance treatment in NBD. Data are very limited because of the small number of cases and the fact that two of the patients still receive infliximab in parallel, nevertheless leflunomide might be an alternative maintenance regimen for NBD.