Cyclosporin for the prevention of disease reactivation in relapsing ANCA-associated vasculitis

Background In patients with ANCA-associated vasculitis the frequent development of relapses after successful initial treatment remains a major therapeutic problem. Thus a long-term prophylactic therapy with low side-effect potential is needed. As recent data suggest an involvement of T cells in the pathogenesis of ANCA-associated vasculitis, the prophylactic value of therapy with low-dose cyclosporin was investigated in seven patients (three with Wegener's granulomatosis, four with microscopic polyangiitis, all with renal involvement) who had developed at least one relapse during cyclophosphamide (CP) treatment or in the first 4 months after the end of CP therapy. Methods After remission had been achieved for 6 months using CP and prednisolone, the CP dose was reduced (3 months 75%, 3 months 50%) and cyclosporin was added concomitantly (dose adjusted to whole blood levels 60-90 ng/ml). Cyclosporin therapy was continued for 1 year after the end of CP treatment. Results During a mean follow-up of 24 months no patient developed a relapse. Two patients developed a herpes zoster infection. No severe bacterial infection occurred. Conclusions These preliminary results indicate that cyclosporin can be successfully used to sustain remission in patients with a relapsing course of ANCA-associated vasculitis and renal involvement.