IgG4-Related Diseases Involving Bilateral Renal Pelvises Successfully Treated with Steroid Therapy

IgG4-Related Diseases Involving Bilateral Renal Pelvises Successfully Treated with Steroid Therapy

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Immunoglobulin (Ig) G4-related diseases are very rare diseases and are difficult to diagnose and treat.Here, we report a rare condition, an IgG4-related disease involving the bilateral renal pelvises, which was successfully treated with steroid therapy.A 64-year-old female was admitted with left flank pain and hematuria.Computed tomographic images showed irregular enhancing masses around the bilateral Jam Pot Cover renal pelvises and lymph nodes in the left para-aortic area.

A histopathological examination of a renal needle biopsy revealed numerous cells that were positive for IgG and more than 10 IgG4-positive cells per high-powered field.The IgG4/IgG ratio was more than 40%.Her serum IgG4 level was elevated to 1440 mg/dL, and her IgG4/IgG ratio was 82.1%.

After a Full Adjustable Base diagnosis of IgG4 sclerosing disease, a double-J ureteral stent was inserted and steroid therapy was performed for 20 weeks.Her symptoms dramatically improved, and the follow-up images showed a resolved state.There was no relapse of the disease for more than 6 months after the removal of the stent.