Retroperitoneal fibrosis

Case contributed by Dr Sherif Mohsen


A man with familial Mediterranean fever, complaining of abdominal and back pain.

Patient Data

Age: 45 years
Gender: Male

A large amalgamated retroperitoneal peri-aortic soft tissue mass/sheet-like lesion. It is seen encasing the aorta and both common iliac arteries, associated with mild compression, yet no evidence of significant stenosis.


Nuclear medicine

(FDG) PET/ CT confirms the mass lesion, showing A large metabolically active amalgamated retroperitoneal paraaortic soft tissue mass/sheet-like lesion, with max.SUV~11.7. 

Case Discussion

A known case of familial Mediterranean fever, presented with retroperitoneal peri-aortic soft tissue mass/sheet-like lesion, that was found to be retroperitoneal fibrosis. 

There are two types: 

  • primary (idiopathic): 2/3 of cases
    • a manifestation of systemic autoimmune or inflammatory diseases
  • secondary: 1/3 of cases
    • most commonly due to medications, neoplasms


  • not useful as a primary diagnostic tool due to low specificity
  • allows detection of remote sites of disease
  • may help guide biopsy and assess treatment response

Top Differential Diagnoses

    • chronic periaortitis
    • retroperitoneal metastases and lymphoma
    • coagulopathic ("retroperitoneal") hemorrhage
    • inflammatory abdominal aortic aneurysms
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    Case information

    rID: 66375
    Published: 19th Feb 2019
    Last edited: 2nd Mar 2019
    System: Vascular
    Inclusion in quiz mode: Included
    Institution: National Institute of diabetes and endocrinology

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