An adult case of cyanotic nephropathy with light chain proximal tubulopathy after partial remission of membranous nephropathy

 

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An adult case of cyanotic nephropathy with light chain proximal tubulopathy after partial remission of membranous nephropathy

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Masayoshi
Ishii
Masayoshi Ishii masayoshi.ishii0505@gmail.com Chiba University Department of Nephrology, Graduate School of Medicine Chiba Japan *
Daisuke Honda dhonda@chiba-u.jp Chiba University Department of Nephrology, Graduate School of Medicine Chiba Japan -
Hanae Wakabayashi hawakaba@chiba-u.jp Chiba University Department of Nephrology, Graduate School of Medicine Chiba Japan -
Katsuhiko Asanuma kasanuma@chiba-u.jp Chiba University Department of Nephrology, Graduate School of Medicine Chiba Japan -
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Cyanotic nephropathy (CN), caused by chronic hypoxemia in cyanotic congenital heart disease (CCHD), typically results in glomerular hyperfiltration and secondary FSGS. In contrast, light chain proximal tubulopathy (LCPT) arises from monoclonal light chain–induced tubular injury. The coexistence of these two rare entities has never been reported.

A 26-year-old man with CCHD presented with nephrotic syndrome. He had first developed nephrotic syndrome at the age of 12, when kidney biopsy revealed membranous nephropathy and CN. Immunosuppressive therapy induced partial remission. At the age of 26, nephrotic-range proteinuria (8 g/gCr) relapsed with urinary M-protein positivity and urinary level of N-acetyl-β-D-glucosaminidase was increased to 52.2 U/L, suggesting renal tubular injury. A second kidney biopsy was performed for diagnostic clarification. 

In the second kidney biopsy, most glomeruli demonstrated mesangial matrix expansion and glomerular hypertrophy. Immunofluorescence staining, kappa light chain depositions were predominantly observed in tubular epithelial cells. Electron microscopy demonstrated irregular granular material within proximal tubular epithelial cells. Therefore, he was diagnosed with CN coexisting with LCPT. Proteinuria decreased to 2 g/gCr with bed rest and dietary restriction and stabilized around 3 g/gCr after initiation of a sodium glucose cotransporter 2 (SGLT2) inhibitor, without immunosuppressive therapy. In CN, a nephrotic level of proteinuria has been reportedly caused by secondary FSGS due to glomerular hyperfiltration. However, the current patient was diagnosed with membranous nephropathy in the first biopsy and LCPT in the second biopsy, respectively, suggesting that various renal diseases can coexist with CN. Therefore, when urinary abnormal findings are detected in CN, kidney biopsy should be considered for making an accurate diagnosis.

We report a case emphasizing that patients with CN can coexist with multiple etiologies of renal diseases such as FSGS, MN and LCPT.

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