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Abstract: PO1718

D-Penicillamine-Induced ANA(+)ANCA(+) Crescentic Glomerulonephritis in Wilson Disease

Session Information

Category: Glomerular Diseases

  • 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix


  • Koga, Shinichiro, Section for Nephrology and Hypertension, Department of Medicine, Tokyo Metropolitan Police Hospital, Tokyo 1868541, Japan

Wilson's disease is an inherited autosomal recessive disorder caused by loss of function of a copper exchanger adenosinetriposphate encoded by ATP7B,which results in impaired biliary copper excretion and accumulation of copper in plasma and tissues.In the kidney,copper accumulation may affect tubular cells,but was never associated with glomelular lesions.Some patients have been reported as crescentic glomerulonephritis with Wilson's disease treated with D-penicillamine1)2).

Case Description

A 45 years old female was consulted to adult nephrology clinic on worsening chronic kidney disease.She had prescribed 800mg metalcaptase for 3 years on compound heterogenous Wilson's disease diagnosed by liver biopsy,Western blotting and gene sequence on ATP7B3).Serum creatinine(Cr) was around 4.5 mg/dl,antimyeloperoxidase(MPO)-ANCA 350 U/ml,antinuclear factors titer 1/640. Both anti-proteinase(PR3) antibody and anti-glomelular basement membrane(GBM) antibody were negative.Renal biopsy specimen show pauci-immune crescentic glomerulonephritis with 7/9 fibrous or fibrocellular crescents,and 2/9 global collaptic sclerosis. Also,mild diffuse interstitial fibrosis was found with lymphoid and other chronic inflammatory cells. No pathological finding was detected on vasculitis.Diagnosed as ANCA-associated glomerulonephritis,methyl-prednisolone(PSL) pulse therapy was given and preceded to oral PSL4).PSL was gradually reduced and terminated after seven years,along with getting MPO-ANCA<3.5 U/ml.Cr was improved to 1.5mg/dl in spite of presence of mild diffuse interstitial fibrosis.Hereafter 15 years,no medication except metalcaptase was given,but regretfully Cr has been deteriorated to 4.5mg/dl,MPO-ANCA~15-25 U/ml. Other paraproteinemia or malignant diseases including multiple myeloma was excluded.Taken together for these 25 years,D-penicillamine-associated interstitial nephritis1)2) has suspected.Oral zinc acetate was once truncated due to gastroenterological side effect,then planned to change to trientene.


The pathogenesis of drug-induced ANCA-associated vasculitis has not been proven.There is a hypothesis that MPO binds to drug metabolites and alters the MPO antigenic property2).Treatment of AAV is usually with mPSL pulse,cyclophosphamide,and/or rituximab.

References:1)Am J Kidney Dis 2007;50:821.2)Clin Nephrol 2016;85:296.3)Kanzo-Hepatology(Tokyo)1996;37Suppl2:116.4)Japan J Nephrol 1998;40:518.