Ormond's Disease - Find Specialists and Information

What is Ormond's disease?

Ormond’s disease is an autoimmune disorder characterized by chronic inflammation behind the abdominal cavity or peritoneum (retroperitoneal). The disease is named after Dr. Ormond, who described it as a distinct clinical entity in 1948.

Ormond’s disease is characterized primarily by the formation of scar tissue (fibrosis). Experts also refer to the disease as retroperitoneal fibrosis.

Similar conditions also occur in the eyes, the thyroid gland, the mediastinum (the space between the lungs and the heart), and the bile ducts. The disease can also affect multiple organs simultaneously. In such cases, it is called multifocal idiopathic fibrosclerosis.

What are the causes of Ormond’s disease?

The triggers for retroperitoneal fibrosis are:

• Infections (tuberculosis, syphilis)
• Connective tissue disorders (lupus erythematosus)
• Radiation therapy
• Asbestos exposure
• Medications

Can Ormond’s disease be prevented?

The most common cause of Ormond’s disease is atherosclerosis of the abdominal aorta. Therefore, smoking, high blood pressure, and high blood lipid levels should be avoided. If the patient has diabetes mellitus or high blood pressure, doctors must carefully manage blood sugar and blood pressure levels.

Care for patients with retroperitoneal fibrosis should take place at a specialized center, where doctors have experience with lifelong immunosuppression. Specialized clinics also have all the necessary diagnostic and therapeutic procedures available.

What are the symptoms of Ormond’s disease?

Symptoms vary depending on the stage of the disease:

• In the early stages of the disease, most patients experience a belt-like pain in the lumbar region. The pain is not affected by body position or physical activity but becomes increasingly severe as the disease progresses.
• This is followed by a general feeling of illness, reduced performance, and weight loss.
• As the inflammation spreads further, it eventually leads to the encasement of the ureter, veins, nerves, and arteries.
• Additional symptoms include flank pain, renal failure, fluid retention in the legs, deep vein thrombosis in the legs, and pain and swelling in the legs.

Pain in the lumbar region associated with Ormond’s disease @ valiantsin /AdobeStock

How is Ormond’s disease diagnosed?

1. Doctors make a preliminary diagnosis based on a CT scan of the abdomen.
2. For further evaluation, doctors perform an MRI. Doctors must also examine the eyes, thyroid, mediastinum, and bile ducts to identify other conditions.
3. The activity of Ormond’s disease is best visualized using (very expensive) positron emission tomography (PET).
4. To rule out other conditions, a biopsy of the inflamed area must be performed. This is the only way doctors can confirm that it is not a tumor.
5. The final diagnosis is made only after all test results are available.

It is particularly important to identify the exact cause of Ormond’s disease. This includes testing for atherosclerosis and rheumatic conditions. If doctors do not know the exact cause, a cure for the patient is nearly impossible.

What does treatment for Ormond’s disease involve?

Ormond’s disease is often an inflammation that almost always originates in the abdominal aorta. The primary focus must therefore be on treating this inflammation. This is the responsibility of the nephrologist or rheumatologist.

The disease often goes unnoticed until the ureter becomes obstructed and urinary retention occurs. As a result, most patients first see a urologist.

In this case, the doctors placed a catheter in the ureter to treat the symptoms. The patient also requires comprehensive treatment with immunosuppressants.

Immunosuppressive therapy is tailored to the disease activity and administered according to the stage of the disease. With effective immunosuppressive therapy, a cure for the disease is possible. The most promising treatment is therapy with mycophenolate.