Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review

Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review

  • نوع فایل : کتاب
  • زبان : انگلیسی
  • مؤلف : Kun-Peng Li & Jian Zhu & Jiang-Lin Zhang & Feng Huang
  • چاپ و سال / کشور: 2011

Description

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. Themean 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.
Clin Rheumatol (2011) 30:601–605 Received: 4 March 2010 / Revised: 29 August 2010 / Accepted: 21 September 2010 / Published online: 19 October 2010 # Clinical Rheumatology 2010
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