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Retroperitoneal fibrosis in the era of immunoglobulin G4-related disease

Yoon Kyung Choi1, Ji Hyun Yang1, Shin Young Ahn2

  • 1Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea.

Kidney Research and Clinical Practice
|February 14, 2019
PubMed
Abstract

Insights

Immunoglobulin G4 (IgG4)-related retroperitoneal fibrosis (RPF) is a significant subset of idiopathic RPF, predominantly affecting older males. Steroid treatment response is similar for both IgG4-related and idiopathic RPF.

Area of Science:

  • Nephrology
  • Immunology
  • Rheumatology

Background:

  • Retroperitoneal fibrosis (RPF) involves fibroinflammatory tissue in the retroperitoneum, often encasing ureters.
  • A subset of RPF is increasingly recognized as part of the spectrum of immunoglobulin G4 (IgG4)-related disease.

Purpose of the Study:

  • To differentiate IgG4-related RPF from idiopathic RPF.
  • To compare clinical and laboratory characteristics and treatment responses between these groups.

Main Methods:

  • Retrospective analysis of 27 idiopathic RPF patients.
  • Categorization of patients based on comprehensive diagnostic criteria for IgG4-related disease.
  • Comparison of clinical, laboratory, and treatment data.

Main Results:

  • 16 out of 27 patients (59.3%) were diagnosed with IgG4-related RPF.
  • IgG4-related RPF patients were predominantly male, older, and had more organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI).
  • No significant difference in response rate to systemic steroid treatment was observed between the groups.

Conclusions:

  • IgG4-related RPF constitutes a substantial portion of cases previously labeled as idiopathic RPF in Korea.
  • Key differentiating features of IgG4-related RPF include male predominance, older age, and higher incidence of postrenal AKI.
  • Further prospective studies are necessary to refine diagnostic criteria and assess long-term outcomes.
Keywords:
Acute kidney injuryImmunoglobulin G4Retroperitoneal fibrosisSteroid treatment

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