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E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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In recent years, steroid reduction regimens combined with other immunosuppressants have been proposed for ANCA-associated vasculitis (AVA) to prevent side effects. The efficacy of Avacopan, one of the candidate combination drugs, for neurological disorders associated with AVA is not well known.
The case was an 82-year-old woman. She had nasal congestion for 8 months and a cough for 6 months, subsequently diagnosed with sinusitis and interstitial pneumonia. One month before admission, she developed bilateral hearing loss, taste disturbance, and facial nerve paralysis, along with lower limb edema, rash, and fever. She was referred to our department. Her serum creatinine level worsened from 0.60 mg/dL to 3.02 mg/dL in about one month, and urinalysis showed hematuria (5-9 /HPF) and proteinuria (1.42 g/gCre). Additionally, p-ANCA was elevated to 37.5 IU/mL, and she was considered to have AVA with neurological disorders and rapidly progressive glomerulonephritis, leading to her admission to our department. Steroid therapy was initiated on the first day of hospitalization, administering 1000 mg of methylprednisolone for three days, followed by prednisolone (PSL) starting at 1.0 mg/kg per day. The steroids were gradually reduced according to the reduction regimen, and Rituximab was administered on the 14th day of hospitalization. Starting from the 16th day, oral Avacopan at 40 mg per day was initiated. After initiation of treatment, her neurological symptoms dramatically improved, and renal function improved to a serum creatinine of 2.41 mg/dL. The BVAS score improved to 6 points, and she was discharged home on the 57th day with PSL at 10 mg per day and Avacopan at 40 mg per day.
AVA is more likely to occur in the elderly, and steroid reduction regimens combined with other immunosuppressants have been proposed to prevent side effects. As candidate combination drugs, Rituximab has a long-term immunosuppressive effect, raising concerns about the risk of infections. Whereas Avacopan offers the advantage of being an oral medication that allows for easy adjustment of dosages. The therapeutic effect of Avacopan for neurological disorders due to AVA has not been clearly established until now. This case suggests that Avacopan may be an effective treatment option for ANCA-associated vasculitis with neuropathy.
This case suggests that a steroid reduction regimen combined with Avacopan may be effective for neurological disorders caused by AVA.
