Possible IgG4-Related Kidney Disease Requiring a Differential Diagnosis of Membranous Lupus Nephritis

Mayumi Yahata; Satoko Takahashi; Izaya Nakaya; Tsutomu Sakuma; Hiroshi Sato; Jun Soma

doi:10.2169/internalmedicine.51.7549

CASE REPORTS

Possible IgG4-Related Kidney Disease Requiring a Differential Diagnosis of Membranous Lupus Nephritis

Mayumi Yahata, Satoko Takahashi, Izaya Nakaya, Tsutomu Sakuma, Hiroshi Sato, Jun Soma

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Keywords: IgG4-related kidney disease, lupus nephritis, IgG4-related tubulointerstitial nephritis, membranous nephropathy

JOURNAL OPEN ACCESS

2012 Volume 51 Issue 13 Pages 1731-1736

DOI https://doi.org/10.2169/internalmedicine.51.7549

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Abstract

A 25-year-old woman presented with fever, arthralgia and proteinuria exhibiting leukopenia, hypocomplementemia, increased serum IgG and IgG4, and positive antinuclear and anti-double-stranded DNA antibodies. Renal biopsy revealed membranous nephropathy with tubulointerstitial nephritis. IgG subclass immunofluorescence revealed intense IgG4 expression in glomeruli, but no expression of IgG2. Observations resembled membranous lupus nephritis with tubulointerstitial nephritis; however, elevated IgG4, low titers of antinuclear and anti-double-stranded DNA antibodies, IgG4-bearing cell infiltration, and characteristic IgG subclass deposition in glomeruli prompted diagnosis of IgG4-related tubulointerstitial nephritis with membranous nephropathy. It is challenging but important to distinguish lupus nephritis from IgG4-related kidney disease.

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