Management of antineutrophil cytoplasmic antibody associated vasculitis

Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS) are grouped together under the term 'Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis' (AAV). Recently, the British Society for Rheumatology and the European League Against Rheumatism have individually published recommendations on the management of AAV. This paper reviews the two recommendations, and the recent advances in the management of AAV. Pattern recognition and clinical suspicion remains the cornerstone for an early diagnosis. The combination of cyclophosphamide and glucocorticoid is the standard of therapy for remission induction. Patients with less severe disease can be treated effectively with less toxic therapies. There is almost no reason to routinely continue using cyclophosphamide beyond 6 months. Long-term remission maintenance is possible with a variety of immunomodulating agents. With increasing survival, the recognition and management of complications like cardiovascular disease, renal failure, malignancy requires long-term follow up and regular screening.