Kidney Research and Clinical Practice 2019 Mar; 38(1): 42-48
Retroperitoneal fibrosis in the era of immunoglobulin G4-related disease
Yoon Kyung Choi1, Ji Hyun Yang1, Shin Young Ahn2, Gang Jee Ko2, Se Won Oh1, Myung Gyu Kim1, Won Yong Cho1, Sang Kyung Jo1
1Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea, 2Department of Internal Medicine, Korea University Guro Hospital, Seoul, Korea
Correspondence to: Sang Kyung Jo, Department of Internal Medicine, Korea University Anam Hospital, 73 Inchon-ro, Seongbuk-gu, Seoul 02841, Korea. E-mail:
Received: June 5, 2018; Revised: September 10, 2018; Accepted: October 24, 2018; Published online: March 31, 2019.
© The Korean Society of Nephrology. All rights reserved.

This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords: Acute kidney injury, Immunoglobulin G4, Retroperitoneal fibrosis, Steroid treatment


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