Retroperitoneal Fibrosis： A Retrospective Clinical Data Analysis of 30 Patients in a 10-year Period

• Published in: Chinese medical journal Vol. 128; no. 6; pp. 804 - 810
• Main Authors: Zhou, Hai-Jiang, Yan, Yong, Zhou, Biao, Lan, Tian-Fei, Wang, Xue-Yan, Li, Chun-Sheng
• Format: Journal Article
• Language: English
• Published: China Medknow Publications Pvt Ltd 20.03.2015; Medknow Publications and Media Pvt. Ltd; Lippincott Williams & Wilkins Ovid Technologies; Department of Emergency Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China%Department of Urology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China%Department of Urology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China%Department of Allergy, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China; Medknow Publications & Media Pvt Ltd; Wolters Kluwer
• Subjects: Abdomen; Adult; Aged; Analysis; Anorexia; Autoimmune diseases; Blood; Cardiovascular disease; Care and treatment; Coronary vessels; Diabetes; Edema; Emergency medical care; Female; Fibrosis; Glucocorticoids - therapeutic use; Hospitals; Humans; Hypertension; Immunoglobulin G - blood; Immunology; Immunosuppression; Immunosuppression; Retroperitoneal Fibrosis; Tamoxifen; Laboratories; Male; Medical imaging; Medical records; Middle Aged; NMR; Nuclear magnetic resonance; Original; Pain; Patients; Prognosis; Retroperitoneal; Retroperitoneal Fibrosis - blood; Retroperitoneal Fibrosis - diagnosis; Retroperitoneal Fibrosis - drug therapy; Retroperitoneal Fibrosis - surgery; Retrospective Studies; Tamoxifen; Tamoxifen - therapeutic use; Treatment Outcome; Urology; China; Retroperitoneal Fibrosis; Tamoxifen; Immunosuppression
• ISSN: 0366-6999; 2542-5641
• DOI: 10.4103/0366-6999.152648

Background： Retroperitoneal fibrosis （RPF） is an uncommon disease that is characterized by development of fibrosclerotic tissues involving retroperitoneal structures. This study aimed to investigate the clinical features of 30 patients with RPF in a single center in Beijing in a 10-year period. Methods： We retrospectively analyzed clinical data on demographic characteristics, clinical manifestations, laboratory findings, radiological findings, modalities of treatments, outcomes and prognosis of 30 patients with RPF. Patients were treated in Beijing Chao-Yang Hospital between January 2003 and December 2013. Results： The mean age of patients with RPF was 56.7 ± 14.4 years. Twenty-three patients were men and seven patients were women. Acute phase reactants were elevated in most patients. Rheumatic factor was positive in 4/25 （16.0%） patients, and antinuclear antibody was positive in 6/22 （27.3%） patients. Elevation oflgG4 was observed in 9/22 （40.9%） patients. The most common type was Ⅰ＋ Ⅲ （17 = 13）, followed by Ⅰ ＋ Ⅱ＋ Ⅲ（n = 12）. Five patients undertook an 18F-fluoro-deoxy-D-glucose positron emission tomography examination and increased uptake was detected in four patients. Eight patients received combination therapy with glucocorticoids and tamoxifen. Surgical intervention treatments included intraureteral double-J stent implantation （n = 26）, percutaneous nephrostomy （n = 2）, open ureterolysis and intraperitonealization of the ureters （n = 5） and laparoscopic ureterolysis and intraperitonealization of the ureters （n = 5）. Three patients underwent hemodialysis because of renal failure. Conclusions： Clinical characteristics of RPF patients in our study are similar to those previously reported. Steroids and immunosuppressive therapy combined with ureterolysis could be a viable choice of treatment for RPF. More prospective, multi-center studies with a longer follow-up are warranted.