title: Retroperitoneal fibrosis in 27 Korean patients: single center experience.
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.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Institute for Immunology and Immunologic Diseases, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: You Jung
authors.initials: YJ
authors.lastname: Ha
authors.forename: Se Jin
authors.initials: SJ
authors.lastname: Jung
authors.forename: Kwang Hoon
authors.initials: KH
authors.lastname: Lee
authors.forename: Sang-Won
authors.initials: SW
authors.lastname: Lee
authors.forename: Soo-Kon
authors.initials: SK
authors.lastname: Lee
authors.forename: Yong-Beom
authors.initials: YB
authors.lastname: Park
mesh_terms: D000208:Acute Disease; D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology / Q000378:metabolism; D001379:Azathioprine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D056910:Republic of Korea; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D012189:Retrospective Studies; D012737:Sex Factors; D014057:Tomography, X-Ray Computed
chemical_list: D000974:Antibodies, Antinuclear; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine