PMID- 38093149
OWN - NLM
STAT- Publisher
LR  - 20231213
IS  - 2192-4449 (Electronic)
IS  - 2192-4449 (Linking)
DP  - 2023 Dec 13
TI  - Early transition to avacopan from glucocorticoids applied during induction 
      therapy for microscopic polyangiitis with rapidly progressive glomerulonephritis.
LID - 10.1007/s13730-023-00841-3 [doi]
AB  - Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic 
      autoimmune disease characterized by necrotizing inflammation of small blood 
      vessels. Glucocorticoids (GC) in combination with rituximab or cyclophosphamide 
      can reduce AAV-related mortality and rescue renal function. However, several side 
      effects associated with these agents, including GC toxicity, are concerning. 
      Avacopan, an inhibitor of the C5a receptor, is now available for AAV treatment 
      and is expected to mitigate GC toxicity. We present a case of 
      myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive 
      microscopic polyangiitis (MPA) with rapidly progressive glomerulonephritis 
      treated with an early switch from GC to avacopan in combination with rituximab 
      during induction therapy. Over a 6-month treatment period, clinical remission was 
      achieved and maintained without infection or elevated liver enzyme levels. 
      Efficacy and safety data regarding avacopan for AAV induction therapy remain 
      limited. Therefore, more case reports are required to clarify the role of 
      avacopan in AAV induction and maintenance therapy. Since the MPO-ANCA titer 
      remained elevated despite the clinical remission of AAV in this case, the ANCA 
      titer may not necessarily be a reliable biomarker for predicting AAV relapse when 
      avacopan is applied as an induction therapy for AAV.
CI  - (c) 2023. The Author(s), under exclusive licence to Japanese Society of Nephrology.
FAU - Miyake, Hiromasa
AU  - Miyake H
AUID- ORCID: 0009-0006-4183-6762
AD  - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Faculty of 
      Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, 
      700-8558, Japan. pijk3kjj@s.okayama-u.ac.jp.
FAU - Tanabe, Katsuyuki
AU  - Tanabe K
AD  - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Faculty of 
      Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, 
      700-8558, Japan.
FAU - Yamaji, Shuhei
AU  - Yamaji S
AD  - Department of Nephrology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, 
      730-8518, Japan.
FAU - Kihara, Takashi
AU  - Kihara T
AD  - Department of Nephrology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, 
      730-8518, Japan.
LA  - eng
PT  - Journal Article
DEP - 20231213
PL  - Japan
TA  - CEN Case Rep
JT  - CEN case reports
JID - 101636244
SB  - IM
OTO - NOTNLM
OT  - Avacopan
OT  - Glucocorticoids toxicity
OT  - MPO-ANCA
OT  - Microscopic polyangiitis
EDAT- 2023/12/14 00:42
MHDA- 2023/12/14 00:42
CRDT- 2023/12/13 23:52
PHST- 2023/09/18 00:00 [received]
PHST- 2023/11/15 00:00 [accepted]
PHST- 2023/12/14 00:42 [medline]
PHST- 2023/12/14 00:42 [pubmed]
PHST- 2023/12/13 23:52 [entrez]
AID - 10.1007/s13730-023-00841-3 [pii]
AID - 10.1007/s13730-023-00841-3 [doi]
PST - aheadofprint
SO  - CEN Case Rep. 2023 Dec 13. doi: 10.1007/s13730-023-00841-3.