Related Experiment Video
Updated: Apr 20, 2026
Unilateral Ureteral Obstruction Model for Investigating Kidney Interstitial Fibrosis
Published on: April 25, 2025
Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach
M L Urban1, A Palmisano1, M Nicastro1
1Unit of nephrology, university hospital, Parma, Italy.
Abstract:
Retroperitoneal fibrosis (RPF) is an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy. RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim-Chester disease. Idiopathic RPF is an immune-mediated disease, which can either be isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibro-inflammatory disorder recently renamed as IgG4-related disease. The differential diagnosis between idiopathic, IgG4-related and secondary RPF is crucial, essentially because the therapeutic approaches - especially of idiopathic vs. secondary RPF - can be dramatically different. This review focuses on the clinical, laboratory and imaging features of the different RPF forms, and also provides an overview of the available treatment options.
Insights
Retroperitoneal fibrosis (RPF) is a rare condition causing ureteral obstruction. Differentiating between idiopathic, IgG4-related, and secondary RPF is vital for effective treatment strategies.
Area of Science:
- Nephrology
- Immunology
- Oncology
Background:
- Retroperitoneal fibrosis (RPF) is a rare condition involving fibrous tissue growth in the retroperitoneum.
- It frequently leads to ureteral entrapment and obstructive uropathy.
- RPF can be idiopathic, or secondary to malignancies, infections, drugs, radiotherapy, or Erdheim-Chester disease.
Purpose of the Study:
- To review the clinical, laboratory, and imaging features of various RPF forms.
- To highlight the importance of differential diagnosis between idiopathic, IgG4-related, and secondary RPF.
- To provide an overview of current treatment options for RPF.
Main Methods:
- Literature review of clinical presentations, diagnostic criteria, and therapeutic approaches for RPF.
- Analysis of differentiating factors between idiopathic RPF, IgG4-related disease, and secondary causes of RPF.
- Synthesis of information on diagnostic tools including imaging and laboratory tests.
Main Results:
- Idiopathic RPF is often immune-mediated, potentially isolated or associated with autoimmune conditions or IgG4-related disease.
- Secondary RPF has diverse etiologies including cancer, infection, and iatrogenic causes.
- Accurate diagnosis is critical as treatment varies significantly between RPF subtypes.
Conclusions:
- Distinguishing between RPF subtypes is essential for guiding appropriate patient management.
- Understanding the underlying cause of RPF dictates the therapeutic strategy.
- This review provides a comprehensive overview for clinicians managing patients with RPF.
More Related Videos
07:38A Multimodal Imaging Approach Based on Micro-CT and Fluorescence Molecular Tomography for Longitudinal Assessment of Bleomycin-Induced Lung Fibrosis in Mice
Published on: April 13, 2018
10:21Mechanistic Insight into the Development of TNBS-Mediated Intestinal Fibrosis and Evaluating the Inhibitory Effects of Rapamycin
Published on: September 12, 2019
Related Concept Videos
01:25Ultrasound II: Endoscopic Ultrasound and FibroScan
Endoscopic Ultrasound (EUS):
01:26Nephrotic Syndrome II : Assessment and Medical Management