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Retroperitoneal Fibrosis

A rare disease characterized by chronic inflammatory fibrous tissue accumulation in the retroperitoneal space.

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 Genel Cerrahi department. Book Appointment →

What is Retroperitoneal Fibrosis?

Retroperitoneal fibrosis (RPF) is a disease characterized by chronic inflammation and fibrosis around the aorta and in the retroperitoneum. It is also known as Ormond's disease.

Most cases are idiopathic (70%), and it is thought to be within the spectrum of IgG4-related disease. Secondary RPF can occur due to malignancy, drugs (ergot derivatives), radiation, infections, and vasculitis.

The most common complication is hydronephrosis and renal failure resulting from ureteral involvement. Diagnosis is made by CT/MRI, and malignancy must always be excluded.

Symptoms

Lower back or flank pain
Abdominal pain
Fatigue and weight loss
Oliguria and urinary symptoms
Leg edema (venous compression)
Varicocele in men
Fever and night sweats

Risk Factors

IgG4-related disease
Smoking
Ergot derivative drugs
Asbestos exposure
Radiotherapy
Autoimmune diseases
Malignancy and infections

When to See a Doctor?

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

  • Chronic flank pain and weight loss
  • Unexplained renal dysfunction
  • Swelling in the legs
  • Progressive fatigue
  • Signs of ureteral obstruction

Treatment Methods

01
DJ stent or percutaneous nephrostomy in ureteral obstruction
02
Steroid therapy (first choice)
03
Steroid-sparing drugs (methotrexate, mycophenolate)
04
Rituximab (in IgG4-related cases)
05
Treatment of secondary cause (drug withdrawal, malignancy)
06
Surgical ureterolysis and intraperitonealization

Which Department to Visit?

You can visit our Genel Cerrahi department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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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.