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Rapidly resolved IgG4-related retroperitoneal fibrosis after steroid pulse therapy

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초록

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

Abstract

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

참고문헌 (13)

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  9. 김현우, "고용량 스테로이드로 치유된 후복막 섬유증식증 2예", Kidney Research and Clinical Practice, 24(6), pp.1017~1021 (2005), 대한신장학회
  10. Fry AC, "Successful use of steroids and ureteric stents in 24 patients with idiopathic retroperitoneal fibrosis:a retrospective study", Nephron Clin Pract, 108, pp.c213~c220 (2008)
  11. Mackworth-Young CG, "A double blind, placebo controlled trial of intravenous methylprednisolone in systemic lupus erythematosus", Ann Rheum Dis, 47, pp.496~502 (1988)
  12. Feduska NJ, "Reversal of renal allograft rejection with intravenous methylprednisolone “pulse” therapy", J Surg Res, 12, pp.208~215 (1972)
  13. Rose BD., "Pathophysiology of renal disease", pp.251~ (1987), McGraw-Hill

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