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Novel trends in monitoring and therapy of ANCA associated vasculitides


Authors: Radim Bečvář
Authors‘ workplace: Revmatologický ústav a Revmatologická klinika 1. LF UK, Praha
Published in: Vnitř Lék 2018; 64(2): 164-168
Category: Reviews

Overview

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.

Key words:
biologicals – biomarkers – eosinophilic granulomatosis with polyangiitis – granulomatosis with polyangiitis – microscopic polyangiitis


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