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Immunoglobulin G4-related Retroperitoneal Fibrosis of the Pelvis

Anthony Dyer1, Peter M Sadow2, Erika Bracamonte3

  • 1Division of Urology, Department of Surgery, The University of Arizona, Tucson, AZ.

Reviews in Urology
|July 11, 2014
PubMed

Insights

Retroperitoneal fibrosis (RPF), a rare condition causing tissue scarring, can occur in the pelvis. This case highlights successful treatment of pelvic RPF with immunoglobulin G4 (IgG4) elevation using corticosteroids.

Area of Science:

  • Urology
  • Immunology
  • Pathology

Background:

  • Retroperitoneal fibrosis (RPF) is a rare condition involving tissue replacement by fibrosis and inflammation.
  • RPF typically affects the retroperitoneum but can present in unusual locations.
  • Immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized as a systemic condition.

Purpose of the Study:

  • To report a rare case of pelvic retroperitoneal fibrosis.
  • To discuss the diagnostic findings, including elevated immunoglobulin G4 (IgG4) levels.
  • To highlight successful treatment outcomes for this presentation.

Main Methods:

  • Clinical presentation of a 68-year-old male patient with pelvic RPF.
  • Diagnostic workup including laboratory tests (CRP, ESR, IgG4).
  • Histopathological examination of biopsy samples.

Main Results:

  • Biopsy confirmed RPF in the pelvic region.
  • Elevated levels of C-reactive protein, erythrocyte sedimentation rate, and immunoglobulin G4 (IgG4) were observed.
  • The patient responded well to systemic corticosteroid therapy.

Conclusions:

  • Pelvic RPF is an uncommon manifestation of retroperitoneal fibrosis.
  • Elevated IgG4 levels suggest a potential link to IgG4-related sclerosing disease.
  • Systemic corticosteroids are an effective treatment for this condition.
Keywords:
Retroperitoneal fibrosisSerum immunoglobulin G4

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