Update on antineutrophil cytoplasmic antibody vasculitis.

PURPOSE OF REVIEW: The purpose of this review is to discuss antineutrophil cytoplasmic antibody (ANCA) vasculitis (microscopic polyangiitis and granulomatosis with polyangiitis) and how we have arrived at our current guidelines and treatment methods. The goal is to offer a greater understanding of major clinical trials in the field, and how they have enabled treatment options which have led to reduced morbidity and improved survival for patients.

RECENT FINDINGS: Our understanding and treatment of ANCA vasculitis has changed tremendously over the past 40 years, from a condition with a high mortality without significant treatment options, to a treatable condition. We have moved from the use of more toxic therapies such as Cyclophosphamide towards more common use of rituximab for both induction and maintenance regimens. More recently, the focus has been on attempting to reduce steroid burden and toxicity. The approval of avacopan (a C5a inhibitor) enables a more rapid steroid taper regimen along with usual induction therapy with rituximab or cyclophosphamide. Readers will have the chance to learn about new targets being evaluated for the treatment of ANCA vasculitis.

SUMMARY: ANCA Vasculitis is complex with varying presentations. Through collaboration among multiple specialists, including nephrologists, rheumatologists, pulmonologists, ENT specialists, and neurologists, we are able to achieve a diagnosis and offer organ and life-saving treatments for our patients.