IgG4-related kidney disease

A rare renal disease occurring in the setting of a systemic IgG4 related disease. Renal manifestations include tubulointerstitial nephritis (TIN), membranous glomerulonephritis with or without TIN, IgA nephropathy, membranoproliferative glomerulonephritis, mesangioproliferative immune complex glomerulonephritis, sclerosing pyelitis, IgG4-related plasma cell arteritis, and hydronephrosis associated with IgG4 related retroperitoneal fibrosis or ureteral inflammatory pseudotumor. Patients present with acute or chronic renal failure and radiographic mass lesion.

Tubulointerstitial nephritis

A form of inflammation of the kidney affecting the interstitium of the kidneys surrounding the tubules.


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(per page)
PMID (PMCID)
27785920
 MALE
A case of IgG4-related tubulointerstitial nephritis and membranous glomerulonephritis during the clinical course of gastric cancer: Imaging features of IgG4-related kidney disease.
Horita S, Fujii H, Mizushima I, Fujisawa Y, Hara S, Yamada K, Inoue D, Nakajima K, Harada K, Kawano M.
Mod Rheumatol. 2019;29(3):542-546.
A case of IgG4-related tubulointerstitial nephritis and membranous glomerulonephritis during the clinical course of gastric cancer: Imaging features of IgG4-related kidney disease.
24842479
 MALE Middle Aged
A case of ANCA-negative renal small-vessel vasculitis with tubulointerstitial infiltration of IgG4-positive plasma cells.
Sakairi T, Okabe S, Hiromura K, Motegi S, Sakurai N, Ikeuchi H, Kaneko Y, Maeshima A, Hirato J, Nojima Y.
Mod Rheumatol. 2016;26(5):780-3.
A renal biopsy showed severe tubulointerstitial nephritis (TIN) with extensive infiltration of IgG4-positive plasma cells, suggesting a diagnosis of IgG4-related kidney disease (IgG4-RKD).
26013281
(4446108)
 FEMALE Middle Aged
IgG4-related kidney disease from the renal pelvis that mimicked urothelial carcinoma: a case report.
Zhang H, Ren X, Zhang W, Yang D, Feng R.
BMC Urol. 2015;15:44.
IgG4-related kidney disease is a comprehensive term for renal lesions associated with IgG4-related disease, which mainly manifests as plasma cell-rich tubulointerstitial nephritis with increased IgG4+ plasma cells and fibrosis.
25371379
 MIXED_SAMPLE Adult
Tubulointerstitial fibrosis in patients with IgG4-related kidney disease: pathological findings on repeat renal biopsy.
Arai H, Hayashi H, Takahashi K, Koide S, Sato W, Hasegawa M, Yamaguchi Y, Aten J, Ito Y, Yuzawa Y.
Rheumatol Int. 2015;35(6):1093-101.
Renal parenchymal lesions in patients with IgG4-related kidney disease (IgG4-RKD) are characterized by tubulointerstitial nephritis with storiform fibrosis and infiltration by high numbers of IgG4-positive plasma cells.
24842825
 FEMALE Middle Aged
A case of IgG4-positive plasma cell-rich tubulointerstitial nephritis in a kidney allograft mimicking IgG4-related kidney disease.
Nishikawa K, Takeda A, Masui S, Kanda H, Yamada Y, Arima K, Morozumi K, Sugimura Y.
Nephrology (Carlton). 2014;19 Suppl 3:52-6.
To our knowledge, this is the first report of IgG4-positive plasma cell-rich tubulointerstitial nephritis mimicking IgG4-related kidney disease after kidney transplantation.
24842825
 FEMALE Middle Aged
A case of IgG4-positive plasma cell-rich tubulointerstitial nephritis in a kidney allograft mimicking IgG4-related kidney disease.
Nishikawa K, Takeda A, Masui S, Kanda H, Yamada Y, Arima K, Morozumi K, Sugimura Y.
Nephrology (Carlton). 2014;19 Suppl 3:52-6.
A case of IgG4-positive plasma cell-rich tubulointerstitial nephritis in a kidney allograft mimicking IgG4-related kidney disease.