NDLI: D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Lee, Yeonhee Lee, Sang Taek Cho, Heeyeon
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.
File Format	HTM / HTML
ISSN	03010430
Issue Number	5
Volume Number	85
Journal	Clinical Nephrology
Language	English
Publisher	Dustri-Verlag Dr. Karl Feistle
Publisher Date	2016-05-01
Publisher Place	Germany
Access Restriction	Subscribed
Subject Keyword	Discipline Nephrology Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Chemically Induced Chelating Agents Adverse Effects Glomerulonephritis Hepatolenticular Degeneration Drug Therapy Penicillamine Adolescent Antibodies, Antineutrophil Cytoplasmic Blood Antibodies, Antinuclear Female Humans Case Reports Journal Article
Content Type	Text
Resource Type	Case study Article
Subject	Nephrology

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