Kidney Res Clin Pract.  2019 Mar;38(1):42-48. 10.23876/j.krcp.18.0052.

Retroperitoneal fibrosis in the era of immunoglobulin G4-related disease

• Choi YK
• Yang JH
• Ahn SY
• Ko GJ
• Oh SW
• Kim MG
• Cho WY
• Jo SK

• KMID: 2447143
• DOI: http://doi.org/10.23876/j.krcp.18.0052

Abstract

BACKGROUND
Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue in the periaortic or periiliac retroperitoneum, where it frequently encases ureters. There is emerging evidence that a subset of this disease is part of a spectrum of multisystemic autoimmune diseases collectively referred to as "immunoglobulin G4 (IgG4)-related disease".
METHODS
We retrospectively analyzed 27 idiopathic RPF patients and identified a subset as IgG4-related RPF, which we categorized according to recently published comprehensive diagnostic criteria. We compared clinical and laboratory characteristics and response to treatment between the two groups.
RESULTS
Of 27 total patients, 16 (59.3%) were diagnosed as having IgG4-related RPF, and these were predominantly male. They were also significantly older and more likely to have other organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI) compared to those with idiopathic RPF. However, there was no difference in response rate to systemic steroid treatment.
CONCLUSION
IgG4-related RPF accounts for a substantial portion of RPF cases previously identified as "idiopathic RPF" in Korea. Clinical and laboratory characteristics of IgG4-related RPF are similar to those of idiopathic RPF except for a striking male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. More comprehensive, prospective studies are needed to clearly distinguish IgG4-related RPF from idiopathic RPF based on clinical manifestation and to further assess treatment response and long-term prognosis.

Keyword

Acute kidney injury; Immunoglobulin G4; Retroperitoneal fibrosis; Steroid treatment

MeSH Terms

Acute Kidney Injury
Autoimmune Diseases
Humans
Hydronephrosis
Immunoglobulins*
Incidence
Korea
Male
Prognosis
Prospective Studies
Rare Diseases
Retroperitoneal Fibrosis*
Retrospective Studies
Strikes, Employee
Ureter
Immunoglobulins