Ormond's disease - Find specialists and information

What is Ormond's disease?

Ormond's disease is an autoimmune disease with chronic inflammation behind the abdominal cavity or peritoneum (retroperitoneal). The disease is named after Dr. Ormond, who described it as an independent clinical picture in 1948.

In Ormond's disease, scar tissue (fibrosis) predominates. Experts also refer to the disease as retroperitoneal fibrosis or retroperitoneal fibrosis.

Similar diseases also occur in the eyes, the thyroid gland, the mediastinum (mediastinum) and the bile ducts. The disease can also affect several organs at the same time. It is then called multifocal idiopathic fibrosclerosis.

What are the causes of Ormond's disease?

The triggers for retroperitoneal fibrosis are

• Infections(tuberculosis, syphilis)
• Mini-tissue diseases(lupus erythematosus)
• radiotherapy
• Exposure to asbestos
• medication

Can Ormond's disease be prevented?

The most common cause of Ormond's disease is vascular calcification of the abdominal aorta. Smoking, high blood pressure and high blood lipids should therefore be avoided. If the patient suffers from diabetes mellitus or high blood pressure, then doctors must adjust the blood sugar and blood pressure values well.

Retroperitoneal fibrosis patients should be cared for in a specialist center: There you will find doctors who have experience with lifelong immunosuppression. All diagnostic and therapeutic procedures are also available in specialist clinics.

What are the symptoms of Ormond's disease?

The symptoms vary with the stage of the disease:

• In the early stages of the disease, most patients show a girdle-shaped pain in the lumbar region. The pain is independent of body position and 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 more and more, it eventually leads to walled-in ureters, veins, nerves and arteries.
• Additional symptoms include flank pain, kidney poisoning, water retention in the legs, leg vein thrombosis and pain and swelling in the leg.

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

How is Ormond's disease diagnosed?

1. Doctors make a suspected diagnosis based on a computer tomography scan of the abdomen.
2. For further clarification, doctors carry out an MRI scan. Doctors must also examine the eyes, thyroid gland, mediastinum and bile ducts to identify other diseases.
3. The activity of Ormond's disease is best visualized by (very expensive) positron emission tomography.
4. A biopsy of the inflammation must be taken to rule out other diseases. This is the only way doctors can ensure that it is not a tumor.
5. The final diagnosis is only made once all the findings are available.

It is particularly important to find the exact cause of Ormond's disease. This also includes arteriosclerosis and rheumatism diagnostics. If the doctors do not know the exact cause, it is almost impossible for the patient to be cured.

What is the treatment for Ormond's disease?

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

The disease often goes unnoticed until the ureter becomes walled off and urinary retention occurs. This is why most patients end up seeing a urologist first.

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

The immunosuppressive therapy depends on the disease activity and is stage-dependent. With good immunosuppressive therapy, the disease can be cured. The most promising treatment is therapy with mycophenolate.