ANCA-associated vasculitides – advances in the diagnosis and treatme

Abstract:

Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) are a group of rare disorders characterized by inflammatory infiltration of the vascular wall, subsequent necrosis of the walls and dysfunction of the affected organs. It is known that ANCA has a role in the pathogenesis of stimulating endothelial activation and migration and adherence of neutrophils to the vascular wall. ANCA associated vasculitis (AAV) comprises three entities: granulomatous polyangiitis (GPA), microscopic polyangitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), which differ according to clinical features, tissue and organ tropism, and different serological prevalences of ANCA. The most commonly affected organs are the skin, respiratory tract, lungs, kidneys and peripheral nerves. In addition to conventional therapy with glucocorticoids and cyclophosphamide, treatment of severe forms of AAV includes rituximab, especially for the relapsing form of the disease. 

Key words:
antineutrophil cytoplasmic antibodies; granulomatosis; inflammation; poliangitis; vasculitis