[ANCA-associated vasculitis]

As demonstrated in the ADVOCATE-trial avacopan allows for a substantial reduction of glucocorticoid (GC) use during induction of remission. A future role of avacopan in the treatment of GPA and MPA is likely. Likewise, the PEXIVAS-trial showed that GC reduction of up to 60 % compared to standard dose was equal effective. The same trial proved no benefit of plasma exchange in addition to standard induction of remission. Plasma exchange therefore is now mostly obsolete.

Rituximab is well established as maintenance therapy in AAV. The optimal duration however is still unknown. The MAINRITSAN 3 study indicates that prolonged use of more than 2 years is beneficial. EGPA:  In line with earlier clinical evidence a GWAS including 534 EGPA patients indicated at last 2 distinct subgroups. The conception of a predominantly "vasculitis" and a more "eosinophilic" phenotype is supported by this genetic evidence and most likely will lead to more differentiated therapies. With the IL5-antibody mepolizumab a first principle targeting the eosinophilic component of EGPA is now available and proved to be clinically beneficial.