ArticleCEN case reports2025
Successful use of avacopan in a case of ANCA-associated vasculitis with treatment-resistant medium-sized vessel involvement.
Article in CEN case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
2 citing papers in PubMed.
- Avacopan for severe pulmonary hemorrhage requiring extracorporeal membrane oxygenation in a patient with MPO-ANCA positive vasculitis.CEN case reports · 2026Article
- Making use of avacopan in clinical practice.Clinical kidney journal · 2026Review
Corrections and comments
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Authors and funding
12 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We report the case of a 75-year-old woman who presented with fever, right back pain, paresthesia in the right extremities, erythema, purpura, and nodules. She had previously initiated dialysis due to rapidly progressive glomerulonephritis and was transferred to our hospital. Imaging studies revealed multiple cerebral and splenic infarcts and hemorrhage encapsulating the right kidney, likely due to microaneurysms in multiple renal arteries. High MPO-ANCA titers were observed, and a skin biopsy revealed granulomatous inflammation affecting medium-sized vessels, leading to a diagnosis of granulomatosis with polyangiitis (GPA) and ANCA-associated vasculitis (AAV) involving medium-sized vessels. Treatment began with intravenous pulse steroid therapy (methylprednisolone 1000 mg/day) and subsequent oral prednisolone (PSL) 40 mg (about 0.8 mg/kg) and intravenous cyclophosphamide (IVCY) at 250 mg. While her symptoms improved, she developed severe infections, including candidemia and febrile neutropenia. Consequently, we combined PSL with the C5a receptor antagonist avacopan, which allowed for PSL tapering and stabilized her disease. This case is significant as no previous reports of avacopan's efficacy in AAV with medium-sized vessel involvement suggest its potential effectiveness in such cases.
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