Related Experiment Video
Updated: Feb 25, 2026
Unilateral Ureteral Obstruction Model for Investigating Kidney Interstitial Fibrosis
Published on: April 25, 2025
Retroperitoneal fibrosis - a report of five cases
Marta Runowska1, Dominik Majewski1, Mariusz Puszczewicz1
1Department of Rheumatology and Internal Diseases, Poznan University of Medical Science, Poland.
Abstract:
Retroperitoneal fibrosis (RPF) is a rare disease, characterized by inflammation and deposition of fibrotic tissue in the vicinity of the abdominal aorta and iliac arteries. We present a report of five patients admitted to our department between January 2014 and February 2017, diagnosed with RPF. Abdominal pain was the most common presenting symptom; however, in one patient, RPF was identified accidentally in routinely performed ultrasonography. In 4 cases, corticosteroids (CS) in combination with azathioprine were applied as first-line therapy, whereas one patient was treated with intravenous methylprednisolone pulses followed by oral CS. In this paper, clinical features as well as laboratory and radiographic findings together with management and treatment outcomes in patients with RPF are discussed. Given the rarity of the condition, it seems important to report every single case of RPF to help establish its management algorithm.
Insights
Retroperitoneal fibrosis (RPF) is a rare condition causing inflammation and tissue buildup near major arteries. This report details five RPF cases, highlighting clinical features, management with corticosteroids and azathioprine, and treatment outcomes.
Area of Science:
- Nephrology
- Vascular Surgery
- Rheumatology
Background:
- Retroperitoneal fibrosis (RPF) is a rare condition characterized by inflammation and fibrotic tissue deposition around the abdominal aorta and iliac arteries.
- RPF can present with diverse symptoms, including abdominal pain, or be incidentally discovered.
- Understanding RPF's clinical spectrum and treatment responses is crucial due to its rarity.
Purpose of the Study:
- To report clinical features, diagnostic findings, and treatment outcomes of five patients diagnosed with retroperitoneal fibrosis (RPF).
- To discuss the management strategies employed for RPF, including corticosteroid and azathioprine use.
- To contribute to establishing a management algorithm for RPF by sharing case experiences.
Main Methods:
- Retrospective case series of five patients diagnosed with RPF between January 2014 and February 2017.
- Review of clinical presentations, laboratory results, radiographic findings, and treatment regimens.
- Analysis of treatment outcomes following interventions such as corticosteroids and azathioprine.
Main Results:
- Abdominal pain was the most frequent presenting symptom in the reported RPF cases.
- Four patients received corticosteroids (CS) combined with azathioprine as initial therapy.
- One patient was treated with intravenous methylprednisolone pulses followed by oral CS.
Conclusions:
- RPF management often involves corticosteroids, sometimes in combination with other immunosuppressants like azathioprine.
- Early diagnosis and tailored treatment are essential for favorable outcomes in RPF.
- Reporting individual RPF cases is vital for advancing the understanding and treatment protocols for this rare disease.
