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Retroperitoneal Fibrosis (Detailed)

Chronic fibroinflammatory disease around aorta and ureters

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Retroperitoneal Fibrosis (Detailed)?

Retroperitoneal fibrosis is a chronic fibroinflammatory process that encases the infrarenal aorta, common iliac vessels, and ureters causing extrinsic compression.

Approximately two-thirds are idiopathic; secondary causes include drugs (methysergide, ergot, beta-blockers), malignancy, infection, and IgG4-related disease.

Diagnosis is based on CT or MRI showing periaortic plaque-like soft tissue, with biopsy reserved for atypical cases or to exclude malignancy.

Bilateral hydronephrosis with renal failure is a frequent presenting feature; prompt drainage prevents permanent renal damage.

Symptoms

Dull lumbar, flank, or lower abdominal pain (most common initial symptom)
Decreased urine output, anuria, or signs of acute kidney injury
Lower extremity edema and venous thrombosis from caval or iliac vein compression
Constitutional symptoms: malaise, weight loss, low-grade fever
Testicular pain or hydrocele from gonadal vein involvement in men
Claudication if iliac arteries are involved

Risk Factors

Male sex (2-3 times more common) and age 40-60
Smoking and asbestos exposure
Use of methysergide, ergot derivatives, beta-blockers, methyldopa
Atherosclerosis with periaortic inflammation
Underlying IgG4-related disease
Prior retroperitoneal radiotherapy or malignancy

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent unexplained back, flank, or abdominal pain with elevated inflammatory markers
  • Decreased urine output or rising creatinine on routine testing
  • Bilateral hydronephrosis on imaging
  • Lower extremity edema with no cardiac cause
  • Recent diagnosis of IgG4-related disease with new symptoms
  • Constitutional symptoms with imaging suggestive of retroperitoneal mass

Treatment Methods

01
Urgent ureteral stenting or percutaneous nephrostomy for obstructive uropathy
02
High-dose corticosteroids (prednisone 0.5-1 mg/kg/day) tapered over 6-12 months
03
Steroid-sparing agents: tamoxifen, mycophenolate mofetil, methotrexate, azathioprine
04
Rituximab for IgG4-related and refractory disease
05
Withdrawal of offending drugs and treatment of secondary causes
06
Long-term surveillance with imaging and renal function for relapse

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dahiliye (İç Hastalıkları) Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.