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[Clinical features and prognostic analysis of retroperitoneal fibrosis in 32 patients]
Le-mei An1, Yu-feng Xu, Zhuo-li Zhang
1Department of Rheumatology and Immunology, Peking University First Hospital, Beijing 100034, China.
Objective:
To analyze the clinical features and imaging manifestations of retroperitoneal fibrosis (RPF) for improving clinical diagnosis and treatment.
Methods:
The medical records of 32 cases from Jul. 2009 to Dec. 2011 with definite diagnosis of RPF were reviewed retrospectively, and the fibro-inflammatory tissue in CT/MRI imagings were classified and measured. The clinical symptoms, the acute phase reactants, kidney function, and radiological imaging,with or without double-J ureteral stenting were observed and compared between prior and post medications and their correlations were also analysed.
Results:
The average age at onset was 60 years, ranging from 30-78, with male-to-female ratio of 1.91:1. Among these 32 cases, the most common presenting manifestations included abdominal pain, abdominal distension, lumbago, loss of body weight, and pitting edema of lower extremities. Hydronephrosis was found by colour doppler untrasonography in 26 (81.25%) patients. RPF was confirmed by typical CT/MRI imagings in all the patients. All of the patients in our study were treated with corticosteroids and in combination with tamoxifen or immunosuppressive agents in most of the patients, of whom 18 patients were followed up for an average of 14 months. After drug therapy, the patients' symptoms were relieved, ESR and CRP droped, renal function improved, and the size of RPF reflected in CT/MRI imagings became smaller significantly. The average time of removal double-J ureteral stenting was 13.1 months and the extubated rate was 80%. The correlation coefficients between changes of ESR or CRP and transverse diameter in CT/MRI imagings were 0.50 or 0.66 (P<0.05) respectively.
Conclusion:
The presenting manifestations of RPF were nonspecific. However, it is extremely important to recognize RPF, because early drug therapy could alleviate symptoms and reduce the acute phase reactants, improve renal function and improve imaging and extubated rate. When compared with the changes between prior and post medications, changes of ESR and CRP had good correlations with CT/MRI imaging changes respectively.
Insights
Retroperitoneal fibrosis (RPF) presents with nonspecific symptoms, but early corticosteroid therapy significantly improves patient outcomes. Treatment alleviates symptoms, enhances renal function, and reduces fibrosis size, as confirmed by imaging.
Area of Science:
- Nephrology
- Radiology
- Inflammatory Diseases
Context:
- Retroperitoneal fibrosis (RPF) is a rare condition characterized by inflammatory tissue in the retroperitoneum.
- Clinical and imaging features are crucial for diagnosis and management.
- Understanding RPF's presentation and treatment response is vital for patient care.
Purpose:
- To analyze the clinical features and imaging findings of retroperitoneal fibrosis (RPF).
- To evaluate the effectiveness of early drug therapy in managing RPF.
- To correlate changes in inflammatory markers with imaging results post-treatment.
Summary:
- Retrospective analysis of 32 RPF cases revealed common symptoms like abdominal pain and hydronephrosis.
- Patients treated with corticosteroids, often with tamoxifen or immunosuppressants, showed symptom relief, improved renal function, and reduced RPF size on CT/MRI.
- Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) levels correlated with imaging changes, indicating treatment efficacy.
Impact:
- Early diagnosis and treatment of RPF can significantly improve renal function and alleviate symptoms.
- Imaging and inflammatory markers serve as reliable indicators of treatment response.
- This study highlights the importance of prompt medical intervention for better patient outcomes in RPF.
