Retroperitoneal fibrosis is a rare condition in which scar-like tissue develops behind the abdominal cavity, potentially squeezing vital organs and causing serious health complications. While most cases have no clear cause, early diagnosis and treatment can help most patients recover and lead normal lives.
What Is Retroperitoneal Fibrosis?
Retroperitoneal fibrosis, also called Ormond’s disease, is an uncommon medical condition that affects a special area of the body called the retroperitoneum. This is the space located behind the abdominal cavity, in the back part of your belly. The condition gets its name from the fact that it causes unusual tissue growth in this hidden area, tissue that resembles scars and becomes thick and fibrous over time.[1]
When retroperitoneal fibrosis develops, inflammation begins in the tissues surrounding the body’s largest artery, the aorta, which carries blood from the heart to the rest of the body. As the disease progresses, this inflammation triggers the formation of thick, fibrous tissue that can gradually wrap around and squeeze nearby organs and blood vessels. The organs most commonly affected include the tubes that carry urine from the kidneys to the bladder, called ureters, as well as the aorta itself, the vena cava (which returns blood to the heart), and the adrenal glands.[1][2]
The disease was first formally described in 1948 by John Ormond, an American urologist, although an earlier case was documented by a French urologist named Joaquin Albarran. The condition develops slowly over time, with symptoms gradually worsening as the fibrous tissue expands and begins to compress surrounding structures.[2]
How Common Is Retroperitoneal Fibrosis?
Retroperitoneal fibrosis is quite rare. Research from the Netherlands suggests that approximately one to two people per 200,000 to 500,000 develop this condition each year. This means it affects only a tiny fraction of the population, making it a disease that many doctors may encounter only occasionally throughout their careers.[4][5]
The disease shows a clear pattern in who it affects. Men develop retroperitoneal fibrosis approximately twice as often as women, though researchers do not fully understand why this gender difference exists. The condition most commonly appears in middle-aged adults, particularly those between 40 and 60 years old. However, it is important to note that retroperitoneal fibrosis can occur at any age, and both women and people outside this age range can also develop the condition.[1][4][7]
Studies indicate that males represent between 54 and 77 percent of all retroperitoneal fibrosis cases, depending on the patient population studied. This predominance among men remains one of the interesting characteristics of the disease that scientists continue to investigate.[7]
What Causes Retroperitoneal Fibrosis?
The underlying cause of retroperitoneal fibrosis remains a mystery in most cases. Approximately 70 percent of people diagnosed with this condition have what doctors call idiopathic retroperitoneal fibrosis, meaning the cause cannot be identified. Medical researchers believe that the immune system may play a significant role in these cases, possibly reacting abnormally to damaged blood vessels or other triggers, even though the exact mechanism is not yet fully understood.[2][5]
Some cases of idiopathic retroperitoneal fibrosis can now be linked to a condition called IgG4-related disease, which was identified relatively recently in 2003. This is an autoimmune disorder where the body’s immune system mistakenly attacks its own tissues, causing inflammation and scarring in various organs throughout the body. The connection to IgG4-related disease has helped scientists understand some previously unexplained cases.[6]
When retroperitoneal fibrosis does have an identifiable cause, several factors may be responsible. Certain medications are known to trigger the condition. These include beta-blockers used for heart disease (such as propranolol, metoprolol, and atenolol), medications for migraine headaches (particularly ergotamine tablets), hydralazine tablets used to control high blood pressure, dopamine antagonists that treat mental health conditions and nausea, and some pain medications.[1][3]
Cancer can also lead to retroperitoneal fibrosis. Both Hodgkin and non-Hodgkin lymphoma may cause the condition, as can other types of cancer that spread to the retroperitoneal area. Sometimes, cancerous tumors in this region can create a reaction in surrounding tissues that resembles retroperitoneal fibrosis.[1]
Infections represent another potential cause. Tuberculosis, a bacterial infection that primarily affects the lungs but can spread to other parts of the body, has been associated with retroperitoneal fibrosis. Other infections that affect the retroperitoneal space may also trigger the condition. Additionally, radiation therapy used to treat cancers in the abdominal or pelvic region can damage tissues and lead to fibrous tissue formation years after treatment ends.[1][3]
Who Is at Risk for Developing Retroperitoneal Fibrosis?
Several factors can increase a person’s likelihood of developing retroperitoneal fibrosis. Understanding these risk factors can help people and their doctors remain vigilant for early signs of the condition.
Age represents a significant risk factor, with the condition occurring most frequently in people between their fourth and sixth decades of life. Being male also increases risk, as men develop the condition about twice as often as women. However, these demographic patterns do not mean that younger people, older adults, or women are immune to the disease.[1]
Exposure to asbestos, a mineral fiber once widely used in construction materials and various industrial applications, significantly raises the risk of developing retroperitoneal fibrosis. The use of tobacco products also substantially increases risk. What makes these two factors particularly dangerous is that when both are present together, they work synergistically, meaning their combined effect is greater than the sum of their individual effects. The good news is that avoiding even one of these risk factors can meaningfully reduce overall risk.[1][2][6]
People with certain autoimmune conditions face elevated risk. Retroperitoneal fibrosis can occur alongside conditions such as autoimmune thyroiditis, ankylosing spondylitis (a type of arthritis affecting the spine), inflammatory bowel diseases like Crohn’s disease and ulcerative colitis, connective tissue diseases, certain types of vasculitis, and conditions affecting other organs such as sclerosing cholangitis (affecting bile ducts) or autoimmune pancreatitis.[6]
A connection to genetics has been identified through the HLA-DRB1 gene, though retroperitoneal fibrosis rarely runs in families. Most cases occur randomly without any family history. On rare occasions, a few members of the same family may develop the condition, but whether it can be truly inherited remains unclear.[3][4][6]
What Are the Symptoms of Retroperitoneal Fibrosis?
The symptoms of retroperitoneal fibrosis vary considerably from person to person, depending on where the fibrous tissue develops, how extensive it becomes, and which organs or blood vessels it affects. Some people may have no symptoms at all and only discover they have the condition when imaging tests are performed for unrelated reasons.[2][7]
Pain is often the first symptom people notice, though it can be difficult to pinpoint exactly where it originates. In early stages, patients typically experience a dull, poorly localized discomfort in the belly, lower back, or sides of the body. This pain usually does not come and go like kidney stone pain; instead, it tends to be constant and gradually worsens over time. Some people describe it as a vague, nagging sensation rather than sharp or severe pain. Men may also feel pain in the testicles or scrotum.[1][5][7]
As the condition progresses and begins to block the ureters, problems with urination emerge. People may notice they are producing less urine than usual, a symptom called low urine output. In severe cases, urination may become difficult or even impossible. Some individuals might experience blood in their urine, though this is less common, or feel an unusual need to urinate frequently at night.[1][3]
When the fibrous tissue affects blood vessels, particularly those supplying the legs, several symptoms may appear. One or both legs might swell noticeably. The affected leg may change color due to poor blood circulation, becoming pale, bluish, or red. Some people experience leg pain or develop signs of deep vein thrombosis, where blood clots form in the deep veins of the legs. Difficulty moving the legs or a feeling of heaviness when walking may also occur.[1][3]
General symptoms often accompany the more specific ones. Many people feel persistently tired or fatigued, even with adequate rest. Weight loss may occur without deliberate dieting, and appetite often decreases. Some individuals develop fevers or experience night sweats. Nausea is another common complaint. These general symptoms, combined with pain, weight loss, and fatigue, frequently occur together as a cluster.[3][5][7]
Men specifically may notice swelling in the scrotum. Joint pains can develop, and some people experience recurrent urinary tract infections. The variety and non-specific nature of these symptoms often mean that retroperitoneal fibrosis takes time to diagnose, as the symptoms could suggest many different conditions.[7]
Can Retroperitoneal Fibrosis Be Prevented?
Preventing retroperitoneal fibrosis entirely is challenging because the underlying cause remains unknown in most cases. However, certain lifestyle modifications and precautions can reduce risk and potentially slow disease progression in those already diagnosed.
The most important preventive measure involves avoiding known risk factors whenever possible. Steering clear of tobacco products is crucial, as smoking significantly increases the risk of developing this condition. For people who currently smoke, quitting represents one of the most effective steps they can take to reduce their risk. Even if someone has already been exposed to other risk factors like asbestos, avoiding tobacco can still substantially lower overall risk.[1]
Minimizing exposure to asbestos is equally important. While asbestos use has been heavily regulated or banned in many countries, older buildings may still contain asbestos-containing materials. People working in construction, demolition, or renovation should follow proper safety protocols when working with older structures. The combination of asbestos exposure and tobacco use creates a particularly dangerous situation, so avoiding both is especially important.[1][2]
For individuals taking medications known to cause retroperitoneal fibrosis, regular communication with healthcare providers is essential. Doctors can often monitor for early signs of the condition or, when appropriate, consider alternative medications. However, people should never stop taking prescribed medications without first consulting their doctor, as the original condition being treated may pose greater risks than the potential for developing retroperitoneal fibrosis.[1]
Maintaining overall cardiovascular health appears particularly important for people with retroperitoneal fibrosis, as blood vessel disease may actually drive the condition in some cases. This includes regular mild exercise, achieving and maintaining an ideal body weight, following a nutritious and balanced diet, and managing conditions like high blood pressure, diabetes, and high cholesterol. These lifestyle factors not only potentially reduce risk but also improve overall health and wellbeing.[7]
For those with autoimmune conditions that increase risk, working closely with rheumatologists or other specialists to manage the underlying disease may help reduce the likelihood of developing retroperitoneal fibrosis as a complication. Regular monitoring and appropriate treatment of autoimmune disorders represents an important preventive strategy for this high-risk group.[6]
How Does Retroperitoneal Fibrosis Affect the Body?
Understanding how retroperitoneal fibrosis changes normal body functions helps explain why the condition causes the symptoms it does and why treatment is so important.
The disease process begins with inflammation in the tissues surrounding the abdominal aorta, typically at the level of the fourth and fifth lumbar vertebrae in the lower back. This inflammation triggers cells called fibroblasts to become overactive. These cells normally help repair injuries by producing collagen and other proteins that form scar tissue. In retroperitoneal fibrosis, however, this process goes into overdrive, creating excessive amounts of thick, fibrous tissue that continues to grow over time.[5]
As this abnormal tissue expands, it begins to wrap around structures in the retroperitoneal space. The tissue acts somewhat like a slowly tightening rope, gradually compressing whatever lies within it. The ureters, being relatively soft tubes positioned close to the aorta, are almost always affected. When the fibrous tissue squeezes these tubes, urine cannot flow normally from the kidneys to the bladder. This causes urine to back up into the kidneys, a condition called hydronephrosis, which makes the kidneys swell.[1][2]
When urine backs up and kidneys swell, kidney function begins to deteriorate. The kidneys normally filter waste products and excess fluid from the blood, maintaining the body’s chemical balance. When they cannot function properly, toxic substances accumulate in the bloodstream, a condition called azotemia. Blood tests reveal elevated levels of urea and creatinine, waste products that healthy kidneys would normally remove. If left untreated, this can progress to complete kidney failure, where the kidneys can no longer maintain life-sustaining functions.[5]
The fibrous tissue can also affect blood vessels. When it surrounds the inferior vena cava, the large vein that returns blood from the lower body to the heart, blood flow becomes obstructed. This causes blood to pool in the legs, leading to swelling and increasing the risk of blood clots forming in the leg veins. The sluggish blood flow creates the perfect environment for deep vein thrombosis, a potentially dangerous condition where clots can break free and travel to the lungs.[4]
Compression of arteries that supply blood to the legs and intestines can cause additional problems. Reduced blood flow to the legs may cause pain when walking, changes in leg color, and in severe cases, tissue damage. If blood supply to the intestines becomes critically reduced, sections of intestinal tissue can die, causing severe abdominal pain and dangerous internal bleeding.[1]
The chronic inflammation associated with retroperitoneal fibrosis affects the whole body systemically. This explains why people often feel generally unwell, fatigued, and may develop fevers. The body’s inflammatory response, which normally helps fight infections and heal injuries, becomes overactive and persistent. Blood tests often show elevated inflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), indicating ongoing inflammation.[5]
Over time, chronic kidney dysfunction can lead to anemia, a condition where red blood cell levels drop below normal. This occurs because healthy kidneys produce a hormone called erythropoietin that stimulates red blood cell production. When kidneys are damaged, they produce less of this hormone, resulting in fewer red blood cells and contributing to the fatigue many patients experience.[1]
High blood pressure frequently develops in people with retroperitoneal fibrosis, affecting approximately half of all patients. This occurs through multiple mechanisms: kidney dysfunction disrupts the body’s normal blood pressure regulation systems, and compression of blood vessels increases resistance to blood flow. The elevated blood pressure can further damage kidneys and other organs if not properly controlled.[5]
In severe, untreated cases, additional serious complications may arise. These include the inability to urinate at all, jaundice (yellowing of the skin and eyes) if bile ducts become involved, nervous system problems if the fibrous tissue affects nerves, complete bowel obstruction requiring emergency surgery, and life-threatening blood clots. These complications underscore why prompt diagnosis and treatment are so critical.[1]