ANCA-associated vasculitis (AAV) - is a group of rare diseases with a total incidence of 46-186 cases per million inhabitants. In all cases, diseases are characterized by inflammation and necrosis of small blood vessels and in many cases anti-neutrophil cytoplasmic antibodies (ANCA) are detected in the blood serum of the patient. The severity of the disease may range from local form to systemic and life-threatening condition and patient’s survival prediction mainly depends on timely and aggressive treatment.
Granulomatosis with polyangiitis (Wegener granulomatosis) is rare and high-mortality disease which occurs approximately for 3-16 per milion persons per year and affects mostly young males. It may involve many different systems, but involvement of the central nervous system occurs only in 7-11% of patients and is considered as threatening condition.
