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Intern Med. 2018 Oct 1;57(19):2873-2877. doi: 10.2169/internalmedicine.0836-18. Epub 2018 Apr 27.

A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis.

Author information

1
Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan.
2
Department of Nephrology, Kyoto City Hospital, Japan.
3
Department of Nephrology, Osaka Red Cross Hospital, Japan.
4
Division of Nephrology, Department of Internal Medicine, Nagoya University, Graduate School of Medicine, Japan.

Abstract

A 58-year-old man with type 1 autoimmune pancreatitis was referred to nephrologists for severe proteinuria. Laboratory data revealed a high serum IgG4 level, hypoalbuminemia, and massive proteinuria, which were compatible with nephrotic syndrome. The renal pathological findings confirmed the diagnosis of secondary membranous nephropathy concurrent with IgG4-related tubulointerstitial nephritis. Despite the improvement of interstitial markers, the proteinuria was refractory to prednisolone, requiring cyclosporine to achieve complete remission. Membranous nephropathy is a rare manifestation of IgG4-related kidney disease. This case shows that the therapeutic response to prednisolone significantly differs between glomerular lesions and interstitial lesions of IgG4-related kidney disease.

KEYWORDS:

IgG-subclass; IgG4-related kidney disease; M-type phospholipase A2 receptor (PLA2R); membranous nephropathy; tubulointerstitial nephritis

PMID:
29709946
PMCID:
PMC6207830
DOI:
10.2169/internalmedicine.0836-18
[Indexed for MEDLINE]
Free PMC Article

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