retroperitoneal fibrosis (RPF)

see also:

• CT abdomen

• 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

• 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

• 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

• La Revue de Médecine Interne 2015: Idiopathic and secondary forms of retroperitoneal fibrosis: A diagnostic approach