Retroperitoneal Fibrosis

Retroperitoneal fibrosis is a rare disease that causes scar-like tissue. This fibrosis happens on organs in the space behind your abdominal (belly) cavity (retroperitoneum). Inflammation also affects these organs, which include your:

• Urinary system
• Aorta
• Vena cava
• Adrenal glands

As fibrosis gets worse, it can surround and squeeze these organs. The most common organs involved in retroperitoneal fibrosis are your two ureters. These take urine from your kidneys to your bladder.

Symptoms like pain and peeing out less urine worsen over time. Your kidneys may also swell.

Signs and symptoms of retroperitoneal fibrosis

Symptoms vary based on where you have abdominal fibrosis and how bad it is. In the early stages, you may feel pain in your belly, lower back or sides. It may be hard to pinpoint the precise location of the pain. If you have testicles, it’s common to feel pain there, as well.

Other symptoms include:

• Difficulty moving your legs
• Swelling in your scrotum
• Leg swelling, pain or color change
• Fatigue
• Low urine output

Some people don’t have symptoms.

Retroperitoneal fibrosis causes

Most cases of Ormond’s disease have an unknown cause. This is called idiopathic retroperitoneal fibrosis. Other times, it comes from:

• Medications
• Cancer, including Hodgkin and non-Hodgkin lymphoma
• Fibroinflammatory disorder
• Infections like tuberculosis
• Radiation therapy
• Tissue damage from trauma or surgery

Medications that can cause this condition

Drugs that can cause retroperitoneal fibrosis include:

• Beta-blockers for heart disease, like propranolol, metoprolol and atenolol
• Dopamine antagonists, which treat mental health disorders and nausea
• Hydralazine tablets for high blood pressure
• Migraine medications, like ergotamine tablets
• Pain medications (analgesics)

Risk factors

Ormond’s disease often occurs in males between 40 and 60 years old. But it can happen in people of all ages and in females.

Other factors that increase your risk include asbestos exposure and tobacco product use. Having both of these can increase your risk even more. Even avoiding one of them can reduce your risk.

Complications of this condition

Advanced fibrosis in your abdomen can lead to:

• Kidney failure
• Low red blood cell count (anemia)
• Inability to pee (anuria)
• High blood pressure
• Jaundice
• Nausea and vomiting
• Nervous system issues
• Tissue death in your intestines
• Unplanned weight loss
• Blood clots

How doctors diagnose retroperitoneal fibrosis

Healthcare providers often diagnose this condition using blood tests and imaging studies. Your provider may use these tests:

• Physical exam, including pressing on your belly to check for abnormal masses
• Blood tests to detect early signs of kidney disease or issues like anemia
• Kidney ultrasound to assess fibrosis, blockage and other issues if symptoms suggest kidney disease
• CT scan, MRI or nuclear medicine imaging to measure fibrosis severity and show how well your kidneys are working. Healthcare providers typically prefer CT scan and MRI.
• Pyelogram, a special X-ray of your urinary system, to show a blockage. Providers use this more often during management of a blockage they identify.

You may need a biopsy if treatments don’t work or if your provider has concerns about cancer.

How is retroperitoneal fibrosis treated?

Treatment includes medicines and surgery. The best care for you depends on the location and extent of the abdominal fibrosis. If your healthcare provider can identify the condition causing this (like cancer), they’ll focus treatment on that condition.

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

• Corticosteroids: These medicines quickly lower inflammation. This stops the scarring from getting worse.
• Immunomodulators: These drugs change immune system activity. They also lower the risk of future fibrosis episodes. These include medications like methotrexate and rituximab.
• Medication management: If fibrosis is due to medications, your healthcare provider may change the drug or dose.

Other treatments for advanced fibrosis are:

• Surgery: You may need this to remove scar tissue and repair damage to nearby tissue.
• Stents: If you have a blockage in one of your ureters, you may need a provider to put in a small, hollow device (ureteral stent) to keep it open.
• Angioplasty or stent placement: This can treat a blood vessel blockage to improve blood flow.

Recovery time

You may feel better after a few days of corticosteroids. Your kidney function should get better within two weeks of starting the medicine. Recovery from other treatments will take longer. Your provider can tell you more based on your case.

When should I see my healthcare provider?

Contact your provider if you have symptoms of retroperitoneal fibrosis, like belly pain. People with mild cases need ongoing visits to check for signs that it’s come back. You may get blood tests and imaging tests every three to six months during the first year. Checkups happen less often over time.

You may want to ask your provider:

• What’s the best treatment for me?
• What can I do to enhance my treatment?
• Do you think my condition will return?

What can I expect if I have this condition?

In general, the prognosis (outlook) for retroperitoneal fibrosis is very good with treatment. Many people make a full recovery. Early detection can give you the best chance of successful treatment. But fibrosis can come back months or years after treatment. If steroids helped you the first time, they’re likely to help you again if your condition comes back.

If cancer causes this condition, the prognosis is typically significantly worse.

Can retroperitoneal fibrosis be prevented?

Because the condition often happens for no known reason, it’s hard to prevent. But it’s a known complication of certain medicines, 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 harder for fibrosis in your abdomen to come back.