Retroperitoneal fibrosis:a clinical and outcome analysis of 33 cases and review of literature
10.3760/cma.j.issn.1007-7480.2013.06.007
- VernacularTitle:腹膜后纤维化33例临床分析并文献复习
- Author:
Hang LIU
;
Weiguo XIAO
- Publication Type:Journal Article
- Keywords:
Retroperitoneal fibrosis;
Therapy;
IgG4-related systemic disease
- From:
Chinese Journal of Rheumatology
2013;(6):383-386
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical features and outcome of retroperitoneal fibrosis (RPF).Methods Thirty-three cases of RPF treated in the Department of Rheumatology and Immunology were analyzed retrospectively,including the clinical characteristics and laboratory data.Results RPF occurred predominantly in elderly men with untypical clinical manifestations such as back pain,abdominal pain and lower limb edema.In laboratory tests,the inflammatory parameters such as erythrocyte sedimentation rate and C-reactive protein increased significantly,and renal function failure was frequent finding in patients with urethral obstruction.All patients had retroperitoneal soft tissue image or urethral obstruction on computed tomography or magnetic resonance imaging,four of which had histological diagnosis of idiopathic RPF.Twenty-three patients received surgical interventions.Eighteen patients received medical treatment alone including corticosteroids,immunosuppressants,and tamoxifen.Nine patients received corticosteroids after surgical intervention.Surgery and medical treatments are effective for idiopathic RPF.Conclusion Image examination is valuable in diagnosis and differential diagnosis of PRF.Pathological diagnosis is the golden standard.Long-term low dose corticosteroids and immunosuppressants may prevent relapse of idiopathic RPF.
