We report a case of IgG4-related disease presenting both tubulointerstitial nephritis\nand retroperitoneal fibrosis causing acute renal failure in a 63-year-old male. He was\nadmitted to our hospital because of acute renal failure requiring emergent hemodialysis.\nComputed tomography showed a soft-tissue density mass with an irregular\nborder in the retroperitoneum. The mass involved bilateral ureters and had caused\nacute renal failure by bilateral hydronephrosis. Because of a history of uveitis and\nhigh IgG4 levels, we considered a diagnosis of retroperitoneal fibrosis, IgG4-related\ndisease. Kidney biopsy revealed IgG4-related kidney disease with interstitial nephritis.\nAfter relief of urinary obstruction by inserting ureteral catheters into the bilateral\nureters, renal function recovered.
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