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Retroperitoneal fibrosis
Martha Tzou1, David J Gazeley2, Peter J Mason3
1University of Wisconsin School of Medicine & Public Health, Madison, WI, USA.
Abstract:
Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication. The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon.
Insights
Retroperitoneal fibrosis (RPF) is a rare inflammatory disease causing a mass that can obstruct ureters and lead to kidney failure. Treatment involves immunosuppressants to manage symptoms and reduce the mass, though relapses can occur.
Area of Science:
- Nephrology
- Vascular Surgery
- Rheumatology
Background:
- Retroperitoneal fibrosis (RPF) is a rare condition characterized by a fibroinflammatory mass.
- This mass can encase vital structures like the abdominal aorta and ureters.
Observation:
- RPF presents with constitutional symptoms, abdominal pain, and often renal insufficiency due to ureteral obstruction.
- Vascular complications such as venous thrombosis and claudication are less common presentations.
Findings:
- Idiopathic RPF is most common, but secondary causes include malignancy and medications.
- Pathophysiology is unclear, but links to periaortitis and IgG4-related disease exist.
Implications:
- Treatment focuses on symptom relief and managing complications from mass effect.
- Immunosuppressants can induce remission, but RPF often follows a relapsing course.
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