retroperitoneal_fibrosis
Table of Contents
retroperitoneal fibrosis (RPF)
see also:
Introduction
- an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy or compression of the IVC
- it is usually detected incidentally on a CT abdomen
- two thirds of cases are idiopathic and are regarded as being immune-mediated - either isolated or with other auto-immune features, and form part of the spectrum of chronic periaortitis
- may develop around an undilated or a dilated aorta, therefore “non-aneurysmal forms” and “peri-aneurysmal forms” of RPF can be distinguished
Primary causes
- idiopathic
- genetic predispositions
- HLA-DRB1*03
- delta 32 (Δ32) polymorphism of the CC-chemokine receptor 5 (CCR5) gene is associated with the aneurysmal form
- environmental factors:
- asbestos exposure (10x odds ratio if also a smoker)
- tobacco smoke
- IgG4-related disease
Secondary causes
- malignancies such as lymphoma
- due to either:
- excessive desmoplastic response of retroperitoneal metastases (e.g., carcinoma of the prostate, breast, colon) or, a primary retroperitoneal tumor (e.g., Hodgkin's and non-Hodgkin lymphomas, inflammatory myofibroblastic tumour, well-differentiated liposarcoma sclerosing variant and various types of sarcomas)
- carcinoids
- infections
- trauma
- radiotherapy
- surgery
- medications / drugs
- ergot alkaloids
- dopamine agonists (e.g., pergolide, methyldopa)
- infliximab, a monoclonal antibody directed against tumor necrosis factor-α (TNF-α)
- etanercept, a soluble receptor that acts as a TNF-α blocker
References
retroperitoneal_fibrosis.txt · Last modified: 2025/06/04 03:19 by wh