[Strategies for effective treatment of ANCA-associated vasculitides with fewer side effects].

Abstract

The pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides is complex; however, a better understanding in recent years has enabled new therapeutic approaches. In recent years priority was given to the minimization of treatment-associated toxicity. For induction of remission of severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), glucocorticoids are used as well as cyclophosphamide and rituximab. The current recommendations enable a more rapid tapering of steroid dose and advise caution in the use of plasmapheresis. Rituximab and azathioprine are available options for maintenance of remission. The choice of medication and duration of remission maintenance are oriented particularly to the risk of recurrence. The importance of low-dose steroids has not yet been finally clarified. New treatment approaches, such as the C5a receptor inhibitor avacopan could enable a minimized steroid treatment in the future. The treatment of eosinophilic granulomatosis with polyangiitis (EGPA) is less evidence-based and consists of glucocorticoids, immunosuppressive agents depending on the severity and increasingly more biologics, e.g. interleukin‑5 blockade. Supportive measures (e.g. vaccinations, infection prophylaxis, cardiovascular risk management) are increasing in importance. Future treatment strategies must take the individual risk (e.g. ANCA subtype, relapse risk) more into consideration for selection and duration of treatment.