J Korean Med Sci.  2011 Aug;26(8):985-990. 10.3346/jkms.2011.26.8.985.

Retroperitoneal Fibrosis in 27 Korean Patients: Single Center Experience

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Institute for Immunology and Immunologic Diseases, Yonsei University College of Medicine, Seoul, Korea. yongbpark@yuhs.ac

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease with unclear etiology, which is characterized by chronic non-specific inflammation of the retroperitoneum. This study was performed to investigate the clinical characteristics, laboratory findings, radiologic findings, treatment and outcome in Korean patients with RPF. We retrospectively reviewed medical records of 27 RPF patients who were admitted to Yonsei University Medical Center between 1998 and 2009. Twenty-two patients (81%) were male. The mean age at diagnosis was 56 yr. Nine patients had identifiable risk factors of RPF and three patients had combined autoimmune diseases. Acute phase reactants were elevated in most patients. Rheumatoid factor was positive in 3 of 16 patients (19%) and antinuclear antibody in 4 of 17 (24%). Five of 6 patients who were taken positron-emission tomography showed positive uptake. Glucocorticoids were used in 16 patients (59%) and four of them received combination therapy with azathioprine. After immunosuppressive treatment, the levels of acute phase reactants dropped, and the size of mass also decreased in most patients. In conclusion, the clinical characteristics of RPF in Korean patients are similar with other series except for higher proportion of male. Some patients with RPF have autoimmune features. The effect of immunosuppressive treatment on RPF is good.

Keyword

Retroperitoneal Fibrosis; Autoimmune Diseases; Immunosuppression

MeSH Terms

Acute Disease
Adult
Aged
Antibodies, Antinuclear/immunology/metabolism
Azathioprine/therapeutic use
Drug Therapy, Combination
Female
Glucocorticoids/therapeutic use
Humans
Immunosuppressive Agents/therapeutic use
Male
Middle Aged
Positron-Emission Tomography
Republic of Korea
Retroperitoneal Fibrosis/*diagnosis/drug therapy/pathology
Retrospective Studies
Sex Factors
Tomography, X-Ray Computed

Cited by  1 articles

Treatment Response to Idiopathic Retroperitoneal Fibrosis-associated Hydronephrosis With a Focus on IgG4/IgG3 Serum Concentration Ratio
Su Jin Choi, Ji Seon Oh, Seokchan Hong, Chang-Keun Lee, Bin Yoo, Bumsik Hong, Yong-Gil Kim
J Rheum Dis. 2021;28(1):38-44.    doi: 10.4078/jrd.2021.28.1.38.


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