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.
Testicular inflammation as a new manifestation of IgG4-associated disease.
de Buy Wenniger LM, Scheltema JM, Verheij J, Beuers U. Urology. 2013;82(2):e15-6.
IgG4-related disease has properties of a systemic disorder but simultaneously is associated with a growing list of organ-specific manifestations including autoimmune pancreatitis, IgG4-associated cholangitis, IgG4-related kidney disease, and IgG4-associated prostatitis.
