Abstract
Children with anti-neutrophil cytoplasmic antibody-associated vasculitis benefit immensely from avacopan as it reduces the requirement for steroids. However, descriptions of adverse drug reactions in children are lacking, and the dosage and follow-up intervals are unclear. A 10-year-old boy with initial granulomatosis and polyangiitis presented with diffuse pulmonary hemorrhage. Rituximab and 30 mg avacopan were administered twice daily as induction therapy following methylprednisolone pulse therapy. However, sudden liver function test abnormalities were observed on day 31 of avacopan treatment, despite liver enzyme levels being within the normal range 5 days earlier. A drug-induced lymphocyte stimulation and various infectious disease tests yielded negative results. Discontinuation of rituximab and avacopan resulted in improved liver function; no change in the Birmingham Vasculitis Activity Score during liver function test abnormalities was observed. Avacopan-associated abnormalities in liver function tests suggest that drug-induced liver injury may occur rapidly in children, and appropriate dosing strategies should be reconsidered.
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The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.
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Acknowledgements
We thank the patient’s parents for their consent to publish this case report and Dr. Aiko Sakai of the Center Hospital of the National Center for Global Health and Medicine for her kind advice in identifying the cause of liver function test abnormalities.
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All authors contributed to the study conception and design. TN mainly drafted the manuscript, data collection, and material preparation; ST and MH contributed to the data collection; KT critically reviewed the manuscript; MM supervised the entire study process. All authors read and approved the final manuscript.
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The study was approved by the Teikyo University Ethical Review Board for Medical and Health Research Involving Human Subjects (approval number 20–195-12). All procedures in this study involving human participants were performed in accordance with the ethical standards of the institutional and/or national research committee and the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.
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Nishino, T., Tomori, S., Haruyama, M. et al. A case of rapid avacopan-induced liver injury in pediatric granulomatosis with polyangiitis. Pediatr Nephrol (2024). https://doi.org/10.1007/s00467-024-06376-8
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DOI: https://doi.org/10.1007/s00467-024-06376-8