D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease
- Authors
- Lee Y.[Lee Y.]; Lee S.T.[Lee S.T.]; Cho H.[Cho H.]
- Issue Date
- May-2016
- Publisher
- DUSTRI-VERLAG DR KARL FEISTLE
- Keywords
- antinuclear antibody; antineutrophil cytoplasmic antibodies; D-penicillamine
- Citation
- CLINICAL NEPHROLOGY, v.85, no.5, pp.296 - 300
- Indexed
- SCIE
SCOPUS
- Journal Title
- CLINICAL NEPHROLOGY
- Volume
- 85
- Number
- 5
- Start Page
- 296
- End Page
- 300
- URI
- https://scholarworks.bwise.kr/skku/handle/2021.sw.skku/36920
- DOI
- 10.5414/CN108763
- ISSN
- 0301-0430
- Abstract
- Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with systemic vasculitis. The pathophysiology of ANCA-associated vasculitis (AAV) has not been clearly proven, and drug-induced ANCA-associated vasculitis has been reported. Wilson's disease is an inborn error of copper metabolism caused by a mutation in the copper transporting gene ATP7B, and traditional treatment is based on copper chelation with agents such as D-penicillamine. There have been rare reports that prolonged D-penicillamine therapy might cause adverse renal events such as membranous nephropathy and minimal change disease, but it is questionable if D-penicillamine induces ANCA-associated vasculitis. We describe 2 patients with Wilson's disease treated with D-penicillamine who presented with ANCA (+) vasculitis and renal involvement. The 2 patients also showed positive results for antinuclear antibody (ANA). Their kidney biopsy findings were compatible with crescentic/necrotizing glomerulonephritis, pauci-immune type. After diagnosis of AAV, D-penicillamine was stopped. Patients were then treated with plasmapheresis and immunosuppressants, including methylprednisolone pulse therapy and intravenous cyclophosphamide. One patient progressed to end-stage renal disease and the other showed persistent proteinuria. These cases suggest that D-penicillamine may induce ANA (+) ANCA (+) vasculitis with severe renal involvement in pediatric patients, and plasmapheresis combined with immunosuppressant should be considered.
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- Appears in
Collections - Medicine > Department of Medicine > 1. Journal Articles
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