Retroperitoneal Fibrosis

What is the retroperitoneum?

Your peritoneum is a layer of tissue that protects most of the organs in your abdomen. The retroperitoneum is a space behind your abdominal cavity.

It includes your:

• Urinary system.
• Kidneys.
• Large vessels that transport blood to and from your legs (inferior vena cava).
• Adrenal glands.

What is retroperitoneal fibrosis (Ormond’s disease)?

Retroperitoneal fibrosis is a rare form of retroperitoneal disease that causes scar-like tissue (fibrosis) over organs in the retroperitoneum. As a progressive disorder, symptoms like pain and low urine output worsen over time.

How does it affect my body?

As fibrosis progresses, it affects the organs and structures in the retroperitoneum. It often affects the ureters, which transport urine from your kidneys to your bladder. In advanced cases, fibrosis may cause life-threatening complications, including kidney failure.

Who gets retroperitoneal fibrosis?

The condition often occurs in men between 40 and 60 years old.

Other factors that increase your risk include:

• Asbestos exposure.
• Smoking or using tobacco products.

What causes Ormond’s disease?

The cause is often unknown (idiopathic). Other times, it’s a complication of:

• Medications.
• Cancer, including Hodgkin and non-Hodgkin lymphoma.
• Infection.
• Radiation therapy.
• Tissue damage due to trauma or surgery.

Which medications cause it?

Drugs such as:

• Beta-blockers for heart disease, such as propranolol, metoprolol and atenolol.
• Dopamine antagonists, such as methyldopa and pergolide, which treat mental health disorders and nausea.
• Hydralazine tablets for high blood pressure.
• Migraine medications, like ergotamine tablets.
• Pain medications, including analgesics and nonsteroidal anti-inflammatories (NSAIDs).

What are the symptoms?

Symptoms vary based on the location of fibrosis and how advanced it is. In the early stages, you may experience pain in your abdomen, lower back or sides. The pain may be general, making it difficult to pinpoint its precise location.

Other symptoms include:

• Difficulty moving your legs.
• Hydrocele, swelling in a man’s scrotum.
• Leg swelling, pain or discoloration.
• Low urine output.

What are the complications of Ormond’s disease?

Advanced retroperitoneal fibrosis causes complications that include:

• Anemia (low red blood cell count).
• Anuria, inability to urinate.
• Itching.
• Jaundice.
• Nausea and vomiting.
• Neurologic complications.
• Tissue death (necrosis) in the intestines.
• Weight loss.

How is retroperitoneal fibrosis diagnosed?

Your assessment may include:

• Physical exam, including pressing on the abdomen to check for abnormal masses.
• Blood tests to detect early signs of kidney disease or complications, like anemia.
• Renal (kidney) ultrasound to assess fibrosis and other issues if symptoms suggest kidney disease.
• CT scan to measure fibrosis severity. Scans also help rule out other issues, including swollen lymph nodes in the groin and tumors.

Will I need a biopsy?

Healthcare providers often diagnose retroperitoneal fibrosis using blood tests and imaging studies. A biopsy may be necessary under certain situations, including:

• Concerns of cancer or an infection.
• Initial treatments are not successful.

What types of treatment might I need?

The therapies that are best for you depend on the location and severity of the fibrosis.

In the early stages of Ormond’s disease, your care may include:

• Corticosteroids: These medications work quickly to quiet the body’s inflammatory response, which stops the progression of fibrosis.
• Immunomodulators: These drugs modify immune system activity. They lower the risk of future fibrosis episodes. Immunomodulators can take months to start working, which is why you may also need corticosteroids.
• Medication management: If fibrosis is a result of medications, your healthcare provider may change the drug or dose.

Other treatments for advanced fibrosis are:

• Surgery: You may need a procedure to remove fibrosis and repair damage to nearby tissue.
• Stents: If there’s a blockage in the ureter, you may need a small hollow device (stent) inserted to keep it open.

How can I prevent retroperitoneal fibrosis?

Because the condition often happens for no known reason, it’s difficult to prevent. But it’s a known complication of certain medications, especially if you’re taking them long-term. If you’re on these drugs, your healthcare provider will keep a careful watch for fibrosis symptoms.

Can treatment lower my risk of a relapse?

If you need surgery, the procedure may include wrapping a layer of fat around the ureter. This makes it difficult for fibrosis to come back.

How soon will I feel better?

You may feel better after a few weeks of corticosteroids or medication management. If you need immunomodulators, stents or surgery, recovery will take longer.

What is the outlook for people with retroperitoneal fibrosis?

Many people make a full recovery. The best chances of successful treatment come with early detection. But fibrosis can come back months or years after treatment.

What’s it like living with Ormond’s disease?

People with mild cases need ongoing monitoring to check for signs of recurrence.

What happens after surgery to correct ureter blockages?

In the days following surgery, healthcare providers will check for signs of hydronephrosis. This complication occurs when your body releases urine that had been accumulating in your kidneys.

When your body releases the trapped urine, it can cause:

• Electrolyte imbalances.
• Dehydration.
• Hypovolemic shock.

A note from Cleveland Clinic

Retroperitoneal fibrosis, also called Ormond's disease, causes scar-like tissue in the back of the abdomen. People taking certain medications have a higher risk of this disease. If you've been diagnosed with Ormond's disease, talk to your provider about any questions or concerns you have. They're there to help you.