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.
A type of glomerulonephritis characterized by diffuse mesangial cell proliferation and the thickening of capillary walls due to subendothelial extension of the mesangium. The term membranoproliferative glomerulonephritis is often employed to denote a general pattern of glomerular injury seen in a variety of disease processes that share a common pathogenetic mechanism, rather than to describe a single disease entity
Membranoproliferative glomerulonephritis with predominant IgG2 and IgG3 deposition in a patient with IgG4-related disease.
Ueki K, Matsukuma Y, Masutani K, Tsuchimoto A, Fujisaki K, Torisu K, Tanaka S, Kiyoshima T, Hisano S, Kitazono T, Tsuruya K. BMC Nephrol. 2015;16:173.
Here, we present the first report of a case of IgG4-related kidney disease and membranoproliferative glomerulonephritis showing the discrepancy in IgG subclasses between the kidney interstitium and glomeruli.
