ANCA-associated small vessel vasculitis: clinical and therapeutic advances

The antineutrophil cytoplasmic antibodies-associated small vessel vasculitides are an encompassing group of diseases defined by serologic and anatomic criteria. Their diverse phenotype is an ongoing diagnostic challenge, and early diagnosis is paramount in optimizing outcomes and minimizing morbidity. Therapies targeted at components of the immune system, both cellular and cytokine, have been assessed as both inducers and sustainers of remission. As current data provide clarity in pathogenesis, treatment options and strategies have been evaluated to improve outcomes. However, challenges remain in limiting disease flares and therapy-associated morbidities, and cures are achieved only in cases in which a clear culprit exists.