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Case report: IgG4-related renal disease co-existing with retroperitoneal fibrosis.


ABSTRACT: A 51-year-old woman visited our clinic for orthopnea, chest discomfort, and facial and leg edema. Acute kidney injury and pleural effusion were diagnosed and she received hemodialysis. This is a rare case of IgG4-related disease diagnosed by renal biopsy in the patient with retroperitoneal fibrosis and severe acute kidney injury, which progressed rapidly and required hemodialysis without markedly elevated serum IgG4 level and urinary tract obstruction. Immunostaining of a kidney biopsy revealed IgG4-related renal disease and magnetic resonance imaging showed retroperitoneal fibrosis in the paraspinal and presacral areas. After 3 days of glucocorticoid treatment, renal function and bilateral pleural effusion were restored. Another 2 months of glucocorticoid treatment decreased the extent of

SUBMITTER: Kim YJ 

PROVIDER: S-EPMC7215037 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature