Abstract
Vasculitides with positivity of autoantibodies to neutrophil leukocytes cytoplasm (ANCA, AAV) belong to primary vasculitides involving small and less commonly medium size blood vessels. Three different clinical types of AAV can be distinguished: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis.
Since these autoantibodies seem to be weak activity biomarkers of AAV new molecules and factors start to come up, e.g. neutrophil extracellular traps NET, several T-lymphocyte subpopulations and different immunoglobulins classes of ANCA. In modern biological therapy rituximab is widely used, for refractory cases intravenous immunoglobulins and antithymocyte globulin are recommended.
The data from clinical trials with alemtuzumab are controversial, but avacopan selective inhibitor of C5a receptor and inhibitor of B-lymphocyte activation factor belimumab are promise for future.