No abstract

Anti-Neutrophil Cytoplasmic Autoantibody (ANCA)-Associated Vasculitides (AAVs) are rare disorders characterized by inflammation of the small blood vessels resulting in ischemia or haemorrhage. The main phenotypes of AAVs are Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA). Although the pathogenesis of these disorders is still incompletely understood, distinct roles for T cells subsets and ANCA autoantibodies were underscored. AAVs present in the head, neck, lung, kidney, skin, heart, eyes, and intestine. The treatment of AAVs aims to induce and maintain remission and rapidly control of the disease activity. Rituximab, the first major alternative to cyclophosphamide plus glucocorticoids therapy, has enhanced the management of AAVs. However, continuous evaluation is constantly needed to manage the uncommon clinical features which may accompany the disease. This review summarizes the major findings of recent studies related to the three kinds of AAVs focusing on new novelties of their pathogenesis, clinical manifestations, and management.