ANCA-Associated Vasculitis

Granulomatosis with polyangiitis (GPA), which was formerly named Wegener's granulomatosis, and microsscopic polyangiitis are associated with autoantibodies against neutrophil cytoplasmatic antigens (ANCA) and are together designated as ANCA-associated vasculitis (AAV) because they also share many clinical features. While granulomatous disease manifestations are by defintion only found in GPA, a vasculitis of predominantly small vessels determines in the clinical picture of the AAV. AAVs are protentially life-threatening diseases, although the prognosis has substantially improved with the last 2 decades due to earlier diagnosis, development of standardised treatment strategies and expert-centre care. Still, in the first year of disease, uncontrolled vasculitis and infections due to immunosuppressive therapy are the major causes of death. In this review we summarise the new nomenclature, symtoms and diagnosis of the AAV and discuss the current treatment recommendations and prognosis.