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[Retroperitoneal fibrosis in adults: Main characteristics and relevance of the diagnostic procedures based on a
B Lioger1, Y Yahiaoui2, J-E Kahn3
1Service de médecine interne, université Francois-Rabelais, hôpital Bretonneau, CHU de Tours, 2, boulevard Tonnelé, 37044 Tours cedex 9, France.
Introduction:
Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the sheathing of retroperitoneal structures by fibro-inflammatory process. It can be either isolated or associated with an underlying disease or condition. In the absence of consistent and consensual approach, the objective of this study was to assess the relevance of diagnostic tests performed during the diagnostic work-up of RPF.
Methods:
Seventy-seven patients were included in this retrospective multicenter study. The diagnosis of RPF was defined by the presence of a thickened circumferential homogeneous tissue unsheathing the infrarenal aorta, excluding peri-aneurysmal fibrosis and a clear evidence of a cancer.
Results:
In 62 cases (80.5%), the RPF was considered as being primary or "idiopathic". Surgical (n=31) or CT-guided (n=9) biopsies of the RPF were performed in half of the patients showing some fibrotic or non-specific inflammatory lesions in 98% of cases. A bone marrow biopsy was performed in 23 patients leading to diagnosis of low grade B cell non-Hodgkin lymphoma in a single patient who also had a monoclonal gammopathy IgM. The systematic search for autoantibodies or serum tumor markers was of no diagnostic value.
Conclusions:
Although the diagnostic procedure was heterogeneous, no cause or associated disease was found in the majority of cases of FRP in this series. In the absence of any clinical or paraclinical evidence suggesting an underlying disease or any atypical features at presentation, a number of non-invasive tests (autoantibodies, tumor markers, bone scintigraphy) and also more invasive diagnostic tests (bone marrow and RPF biopsies) seem of little relevance.
Insights
Retroperitoneal fibrosis (RPF) diagnosis often lacks a clear cause. Most diagnostic tests, including biopsies and autoantibody screening, offer little value in identifying underlying conditions for RPF.
Area of Science:
- Nephrology
- Rheumatology
- Oncology
Background:
- Retroperitoneal fibrosis (RPF) is a rare condition involving fibro-inflammatory sheathing of retroperitoneal structures.
- RPF can be idiopathic or secondary to other diseases.
- A standardized diagnostic approach for RPF is lacking.
Purpose of the Study:
- To evaluate the diagnostic utility of various tests in the work-up of retroperitoneal fibrosis.
- To determine the relevance of invasive and non-invasive diagnostic procedures for RPF.
Main Methods:
- Retrospective multicenter study including 77 patients diagnosed with RPF.
- RPF diagnosis defined by specific imaging criteria, excluding cancer-related fibrosis.
- Analysis of data from biopsies (RPF, bone marrow) and serological tests (autoantibodies, tumor markers).
Main Results:
- 80.5% of RPF cases were idiopathic.
- RPF biopsies showed non-specific inflammatory or fibrotic lesions in 98% of cases.
- Bone marrow biopsy identified one case of lymphoma; autoantibodies and tumor markers were not diagnostically useful.
Conclusions:
- No underlying cause or associated disease was identified in the majority of RPF cases.
- Non-invasive tests (autoantibodies, tumor markers, bone scintigraphy) and invasive tests (biopsies) have limited relevance for RPF diagnosis.
- Diagnostic procedures for RPF were heterogeneous, with limited etiological findings.
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