IgG4-related retroperitoneal fibrosis with acute kidney injury: a case report and literature review

Rheumatol Int. 2023 Nov;43(11):2141-2153. doi: 10.1007/s00296-023-05402-6. Epub 2023 Aug 5.

Abstract

IgG4-related retroperitoneal fibrosis is a rare cause of renal dysfunction that usually manifests as obstructive nephropathy (sometimes with extrarenal manifestations). Due to the non-specific symptoms at the onset of the disease and often latent course, special laboratory and instrumental examination methods are usually needed to establish a diagnosis. The article describes a clinical case of a relapse of IgG4-related retroperitoneal fibrosis in a 53-year-old patient, who developed bilateral ureterohydronephrosis with postrenal acute kidney injury after the withdrawal of glucocorticoid therapy. The patient underwent bilateral percutaneous nephrostomy and resumed glucocorticoids at a dose of 30 mg/day. Postobstructive diuresis was 22 L. Treatment resulted in a complete normalization of the creatinine level and transient hypokalemia, which was eliminated with potassium medications. At the final stage of the treatment, bilateral stenting of both ureters was performed with a tapering of glucocorticoids to 5 mg per day with CT control of the retroperitoneal space after 5 months. A clinical case demonstrates that an interruption of glucocorticoid treatment in patients with IgG4-related retroperitoneal fibrosis can lead to ureterohydronephrosis with the development of acute kidney injury. In such cases, stenting of the ureters could be considered an optimal therapeutic option.

Keywords: Clinical case; Glucocorticoids; Hydronephrosis; IgG4-related disease; Retroperitoneal fibrosis; Ureteral obstruction.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Acute Kidney Injury* / diagnosis
  • Acute Kidney Injury* / etiology
  • Acute Kidney Injury* / therapy
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulin G / therapeutic use
  • Middle Aged
  • Retroperitoneal Fibrosis* / complications
  • Retroperitoneal Fibrosis* / diagnosis
  • Retroperitoneal Fibrosis* / drug therapy

Substances

  • Glucocorticoids
  • Immunoglobulin G