Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review.
The objective of this study is to describe the clinical characteristics of patients with idiopathic retroperitoneal fibrosis (RPF). A retrospective analysis of patients diagnosed with idiopathic RPF in Chinese People's Liberation Army General Hospital was conducted. Sixty-one patients with idiopathic RPF were identified. Male to female ratio was 3.7: 1. The mean age was 55.7 years. One patient had polymyositis and two patients had a family history of idiopathic RPF. Back pain and abdominal pain are the most common symptoms. Forty-seven patients (77.1%) had retroperitoneal soft tissue shadow or ureteral obstruction on computed tomography or magnetic resonance imaging. Thirty-eight patients (62.3%) had histological diagnosis of idiopathic RPF. Thirty-four patients (55.7%) received surgical interventions. Five patients (8.2%) had palliative percutaneous nephrostomy. Ten patients (16.4%) received medical treatment alone including corticosteroids, immunosuppressants, and tamoxifen. Four patients (6.6%) received corticosteroids after surgical intervention. Two patients (3.3%) required renal dialysis. There were two cases of death. Surgery and medical treatments are effective for idiopathic RPF. In conclusion, long-term low dose corticosteroids and immunosuppressants may prevent relapse of idiopathic RPF.
Department of Rheumatology, Chinese People's Liberation Army General Hospital, 28 Fuxing Road, Beijing, 100853, China.
Kun-Peng
KP
Li
Jian
J
Zhu
Jiang-Lin
JL
Zhang
Feng
F
Huang
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001011:Aorta / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D017285:Polymyositis; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D014513:Ureter / Q000473:pathology; D014517:Ureteral Obstruction / Q000628:therapy; D014682:Vena Cava, Inferior / Q000473:pathology