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

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Abstract

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.

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Acknowledgments

The authors would like to thank Dr. Li Z.E. for his revision of the English language used in the manuscript.

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Authors and Affiliations

  1. Department of Rheumatology, Chinese People’s Liberation Army (PLA) General Hospital, 28 Fuxing Road, Beijing, 100853, China

    Kun-Peng Li, Jian Zhu, Jiang-Lin Zhang & Feng Huang

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  1. Kun-Peng Li
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  2. Jian Zhu
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  3. Jiang-Lin Zhang
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  4. Feng Huang
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Corresponding author

Correspondence to Feng Huang.

Additional information

Kun-peng Li and Jian Zhu have contributed equally to this article.

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Li, KP., Zhu, J., Zhang, JL. et al. Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review. Clin Rheumatol 30, 601–605 (2011). https://doi.org/10.1007/s10067-010-1580-6

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  • DOI: https://doi.org/10.1007/s10067-010-1580-6

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