Immunoglobulin A nephropathy secondary to Wilson ’s disease: a case report and literature review

AbstractImmunoglobulin A nephropathy is the most common primary glomerulonephritis worldwide, and it can be associated with liver disease. However, cases of Immunoglobulin A nephropathy secondary to Wilson ’s disease are very rare. A 20-year-old Japanese man presented with microscopic hematuria, proteinuria, and renal dysfunction. A renal biopsy showed mesangial cell proliferation, immunoglobulin A deposition, and electron-dense deposit in the mesangial areas, all of which are consistent with Immuno globulin A nephropathy. Computed tomography of the abdomen showed liver atrophy and splenomegaly, and the diagnosis of Wilson’s disease was confirmed with decreased serum ceruloplasmin levels, increased urinary copper excretion, Kayser–Fleischer rings and copper deposition in the liver biopsy. T he patient was treated successfully with trientine hydrochloride and zinc acetate and showed improvement in renal manifestations. Wilson’s disease is a rare cause of secondary Immunoglobulin A nephropathy. We recommend that Wilson’s disease should be considered the cause of secondary Immunoglobu lin A nephropathy in juvenile patients with hematuria, proteinuria, and splenomegaly and suggest measuring the serum ceruloplasmin concentrations, urinary copper excretion, and evaluating Kayser–Fleischer rings in these patients.
Source: CEN Case Reports - Category: Urology & Nephrology Source Type: research