CEN Case Reports

, Volume 8, Issue 1, pp 61–66 | Cite as

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

  • Yoshinosuke ShimamuraEmail author
  • Takuto Maeda
  • Yufu Gocho
  • Yayoi Ogawa
  • Kunihiko Tsuji
  • Hideki Takizawa
Case Report


Immunoglobulin 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 Immunoglobulin 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. The 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 Immunoglobulin 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.


Wilson’s disease IgA nephropathy Trientine hydrochloride Zinc acetate 


Compliance with ethical standards

Conflict of interest

The authors have declared that no conflict of interest exists.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee at which the studies were conducted (IRB approval number: 2017−126) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  1. 1.Department of NephrologyTeine Keijinkai Medical CenterSapporoJapan
  2. 2.Hokkaido Kidney Pathology CenterSapporoJapan
  3. 3.Center for Gastrointestinal DiseasesTeine Keijinkai Medical CenterSapporoJapan

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