Key advances in the clinical approach to ANCA-associated vasculitis.

Abstract

The updated nomenclature for vasculitis defines this varied group of disorders by aetiology, specific features of pathogenesis and clinical symptoms; diagnostic and classification criteria for clinical practice are in development. Here, I review some important advances in the management of vasculitis within the category of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which encompasses microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The clinical approach to the management of the patient with AAV should include testing for ANCA specificity; proteinase 3 (PR3)-specific ANCAs are most often associated with GPA, whereas myeloperoxidase (MPO)-ANCAs are usually associated with MPA. Also important to the management of AAV is an assessment of the disease stage and severity, to enable tailored treatment based on an algorithm derived from controlled-trial data. Remaining questions pertain to the dosage and duration of corticosteroid treatment, the selection of patients for, and duration of, maintenance treatment after induction of remission, and the identification of safer and more effective therapies than are currently in use. Outcome measures should assess not only disease activity, but also damage and quality of life. Infections, cardiovascular events and malignancies also contribute to outcome, and their prevention should therefore be part of the clinical approach to managing patients with AAV.