PMID- 36545762 OWN - NLM STAT- MEDLINE DCOM- 20230426 LR - 20230814 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 19 IP - 5 DP - 2023 May TI - Avacopan for the treatment of ANCA-associated vasculitis: an update. PG - 461-471 LID - 10.1080/1744666X.2023.2162041 [doi] AB - INTRODUCTION: Glucocorticoids (GC) have been part of the standard treatment of anti-neutrophil cytoplasm autoantibodies (ANCA)-associated vasculitides (AAV) for more than 60 years. Various therapeutic advances have occurred over the past 2 decades and led to a significant reduction of GC exposure, but most patients still have to suffer from complications of GC, including infections, metabolic abnormalities, and cardiovascular morbidity. In 2007, activation of the complement pathway was demonstrated to play a role in the pathogenesis of AAV. Avacopan, an oral competitive inhibitor of the C5a receptor (C5aR1, CD88), was then developed, with an additional aim to decrease the use of GC. AREAS COVERED: In this article, we briefly summarize the rationale for targeting the complement pathway in AAV, and review relevant findings from pre-clinical, phase I, II, and III studies, subsequent and more recent case reports and series on the efficacy and safety of avacopan. EXPERT OPINION: Based on the results of these studies, avacopan was approved in most countries since late 2021, as an adjunctive induction treatment for patients with AAV. Several newer questions now are pending answers, including as to how avacopan should be used in real-world practice, beyond how it was given in the original clinical trials. FAU - Osman, Mohammed AU - Osman M AD - Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada. FAU - Cohen Tervaert, Jan Willem AU - Cohen Tervaert JW AD - Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada. FAU - Pagnoux, Christian AU - Pagnoux C AD - Vasculitis clinic, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada. LA - eng PT - Journal Article DEP - 20221226 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 RN - O880NM097T (avacopan) RN - 0 (Aniline Compounds) RN - 0 (Nipecotic Acids) RN - 0 (Glucocorticoids) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) SB - IM EIN - Expert Rev Clin Immunol. 2024 Mar;20(3):321. PMID: 37578317 MH - Humans MH - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis MH - Aniline Compounds/adverse effects MH - Nipecotic Acids/adverse effects MH - Glucocorticoids/therapeutic use MH - Antibodies, Antineutrophil Cytoplasmic OTO - NOTNLM OT - ANCA OT - avacopan OT - complement OT - glucocorticoids OT - vasculitis EDAT- 2022/12/23 06:00 MHDA- 2023/04/26 06:41 CRDT- 2022/12/22 03:03 PHST- 2023/04/26 06:41 [medline] PHST- 2022/12/23 06:00 [pubmed] PHST- 2022/12/22 03:03 [entrez] AID - 10.1080/1744666X.2023.2162041 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2023 May;19(5):461-471. doi: 10.1080/1744666X.2023.2162041. Epub 2022 Dec 26.