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Idiopathic Retroperitoneal Fibrosis.
Augusto Vaglio1, Federica Maritati2
1Nephrology Unit, University Hospital, Parma, Italy augusto.vaglio@virgilio.it.
Journal of the American Society of Nephrology : JASN
|February 11, 2016
Summary
Idiopathic retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory condition affecting the retroperitoneum. Diagnosis involves imaging, and treatment includes immunosuppressants, with rituximab showing promise for difficult cases.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Idiopathic retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory disease.
- It affects the abdominal aorta, iliac arteries, and surrounding retroperitoneum.
- RPF can be linked to IgG4-related disease and other autoimmune conditions.
Purpose of the Study:
- To review the current understanding of idiopathic retroperitoneal fibrosis.
- To highlight diagnostic methods and treatment strategies.
- To discuss emerging therapies for RPF.
Main Methods:
- Review of existing literature on idiopathic retroperitoneal fibrosis.
- Analysis of diagnostic modalities including CT, MRI, and PET.
- Evaluation of treatment outcomes with glucocorticoids, immunosuppressants, and biologics.
Main Results:
- RPF frequently causes ureteral obstruction and renal failure.
- Renal complications include stenosis, atrophy, and glomerulonephritis.
- Environmental and genetic factors influence RPF susceptibility.
Conclusions:
- RPF diagnosis relies on advanced imaging techniques.
- Treatment focuses on relieving obstruction and inducing regression.
- Biologic therapies like rituximab offer new hope for refractory RPF cases.
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