A CASE OF VANISHING BILE DUCT SYNDROME (VBDS) CAUSED BY AVACOPAN (AVA) ADMINISTRAION FOR TREATMENT OF MICROSCOPIC POLYANGIITIS (MPA)

 

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A CASE OF VANISHING BILE DUCT SYNDROME (VBDS) CAUSED BY AVACOPAN (AVA) ADMINISTRAION FOR TREATMENT OF MICROSCOPIC POLYANGIITIS (MPA)

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Michihisa
Sugita
Michihisa Sugita mitihisa.sugith@gmail.com Central Japan International Medical Center Nephrology Minokamo city Japan *
Yuya Kubota kubota.agate.2810@gmail.com Central Japan International Medical Center Nephrology Minokamo city Japan -
Tomomi Ishihara itomomi213213@gmail.com Central Japan International Medical Center Nephrology Minokamo Japan -
Sumihisa Ono onos@aichi-med-u.ac.jp Central Japan International Medical Center Nephrology Minokamo Japan -
Kanyu Miyamoto k-miyamoto@cjimc-hp.jp Central Japan International Medical Center Nephrology Minokamo Japan -
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Avacopan (AVA) is widely used in remission induction therapy for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, several cases of vanishing bile duct syndrome (VBDS) after AVA administration were reported, especially in Japan. Therefore, further studies are required to reveal relevance of AVA to VBDS.

 

A 42-year-old woman consulted a doctor at a nearby medical clinic, presenting with fever and general malaise. She was referred to our hospital because of renal dysfunction, proteinuria and urinary occult blood. In our medical examination, purpuras were found in her legs and myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was positive. She was hospitalized for a renal biopsy and immunosuppressive treatment. Renal pathological findings showed crescentic glomerulonephritis and fibrinoid necrosis. She was diagnosed microscopic polyangiitis (MPA) and treated with 1,000mg/day methylprednisolone (mPSL) for 3days, followed by 40mg/day prednisolone (PSL). A week after PSL administration, AVA and rituximab (RTX) were added. A month later, although MPA remained in remission, she complained of recurrence of general malaise. 40 days after AVA administration, the levels of bilirubin and liver enzyme (GOT, GPT, γ-GTP) were elevated and jaundice apperred. AVA and RTX were discontinued. A liver biopsy revealed VBDS. She was treated with ursodeoxycholic acid and glucocorticoid (including 1,000mg/day mPSL). For liver injury due to AVA, mycophenolate mofetil (MMF) was added. However, liver function didn’t recover. Although plasma exchange (six times in two weeks) was done, it did not work. Therefore, we referred her to university hospital for consideration of liver transplantation.

We experienced a case of VBDS caused by AVA administration for treatment of MPA. When we are planning to dose the patients to AVA during remission induction therapy for MPA, we should pay attention to risks of liver injury and consider frequent laboratory follow-ups.

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