Treatment of acute c-ANCA-positive vasculitis with mycophenolate mofetil

Acute cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-positive vasculitis is usually treated with cyclophosphamide and corticosteroids. The incidence of cyclophosphamide-induced lung injury, a potentially life-threatening event, is about 1%. We report on a patient with a history of cyclophosphamide-induced lung injury 2 months after initial treatment of systemic c-ANCA-positive vasculitis. Six months later, the patient presented with acute renal failure caused by an acute relapse of vasculitis. Mycophenolate mofetil (MMF) is a potent immunosuppressive drug that recently has been shown to be effective in the maintenance therapy of c-ANCA-positive systemic vasculitis. With the patient's informed consent, we started treatment with MMF in combination with corticosteroids. Subsequently, anti-proteinase-3-titer (anti-Pr3-titer) returned to normal and renal function improved. In conclusion, MMF in combination with corticosteroids may be useful in the treatment of acute c-ANCA-positive vasculitis.