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.
Sudden loss of renal function, as manifested by decreased urine production, and a rise in serum creatinine or blood urea nitrogen concentration (azotemia).
IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia.
Wong ETY, Lahiri M, Teh M, Leo CCH. Case Rep Nephrol Dial. 2019;9(2):49-54.
Here we report the case of a man with interstitial nephritis presenting with acute kidney injury and hypocomplementemia but normal serum IgG4 level and provide a literature review of IgG4-related kidney disease.
