Abstract

ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAV are a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Consequently, a similar therapeutic approach has been developed. A new nomenclature has been recently proposed, with AAV including "granulomatosis with polyangiitis" (GPA, formerly Wegener' s granulomatosis), "microscopic polyangiitis" (MPA), "eosinophilic granulomatosis with polyangiitis" (EGPA, formerly Churg-Strauss disease) and renal limited vasculitis. Research on AAV has shown significant advances in the last two decades, including advances in the knowledge of pathogenic mechanisms such as the pro-inflammatory role of ANCA, AAV murine models and genetic links. Evidence on previous immunosuppressive therapies has improved significantly and new, promissory drugs have been introduced, Rituximab being the most important. We review the advances of pathogenic mechanisms and treatment for these diseases.