Ormond's disease is a chronic aseptic inflammation of the connective and adipose tissue of the retroperitoneal space, which causes compression of the tubular anatomical formations (lymphatic and blood vessels, ureters) located there.
Retroperitoneal fibrosis (another name for the disease) is a type of systemic idiopathic fibrosis, which also includes mediastinal fibrosis, Riedel struma, sclerosing cholangitis, Peyronie's disease and others. All of the above are local manifestations of the same disease.
Prevalence
Fortunately, Ormond’s disease is extremely rare: only one case in two hundred thousand people. As a rule, it is detected in men from thirty to sixty years old. Women of the same age category are sick twice less. Mortality is low, and in most of the cases described it was caused by complications, and not by the disease itself.
Most often, the ureters, which are well covered with subcutaneous fat, fall under the compression effect. They find themselves in a dense narrow case and, due to its rigidity, cease to fulfill their function.
Classification
Clinicians isolate Ormond's primary and secondary disease. Primary, or idiopathic fibrosis, occurs on its own, for unknown reasons. Scientists have several explanations about this:
- breakdown of genes responsible for the structure of connective tissues;
- specific manifestation of autoimmune aggression;
- inflammatory changes.
Secondary retroperitoneal fibrosis is associated with a change in tissue characteristics due to a previous long-term illness. This may be a chronic infection (or carriage of a pathogen), connective tissue disease, and others.
Causes
As mentioned earlier, the causes of Ormond's disease are not known for certain. There are several theories trying to explain the changes in the body.
- Inflammatory As a result of prolonged local edema of tissues, impregnation with fibrin and other acute phase proteins, they become stiff and inactive.
- Immune Due to a malfunction in the protective systems, the body begins to produce antibodies against its own tissues, injures them and replaces these areas with fibrin in response to damage.
- Gene. Damage to the site of the gene responsible for the structure of connective tissue. It manifests itself in a change in the structure of retroperitoneal fiber.
A number of researchers suggest that retroperitoneal fibrosis can be attributed to a number of true collagenoses. There are certain factors that contribute to the manifestation of the disease. These include oncological diseases, hepatitis, pancreatitis, sclerotic changes in adipose tissue in diseases of the digestive tube and female genital organs, tuberculous damage to the bones of the spine, massive hematomas, vasculitis. In addition, chemotherapy courses and drugs that relieve migraine attacks can also contribute to the formation of fibrosis. But in most cases, the trigger factor is not possible to establish.
Pathogenesis
For the most part, retroperitoneal fibrosis (Ormond disease) begins with the appearance of densified areas at the level of the fourth to fifth lumbar vertebra. The iliac arteries and ureters are located there.
Over time, the lesion area expands and, capturing more fiber, descends to the cape of the sacrum, and also to the sides to the gates of the kidneys. In almost half the cases, this process is two-way. Coarse connective tissue becomes comparable in density to wood. It also occurs around the aorta, retroperitoneal lymph nodes, arteries and veins. These anatomical structures are compressed and contracted, their patency worsens over time.
Violation of the patency of the ureters leads to stagnation of fluid in the kidneys, hydronephrosis, chronic inflammation and, as a result, chronic renal failure. In rare cases, intestinal obstruction or obstruction of large vessels joins this.
Symptoms
There are no signs that would uniquely characterize Ormond's disease. It all depends on the stage of the process, its prevalence, activity and specific characteristics of the human body. The duration of the latent course of the disease can vary from two months to eleven years.
All symptoms of Ormond disease can be divided into three periods, based on the time of their appearance:
- the onset of the disease;
- increase in the number of fibrous fibers;
- Fibrin “contraction” and compression.
The patient comes to the local doctor with complaints of dull, aching constant pain in the back or sides. Unpleasant sensations can spread to the lower abdomen, inguinal region, to the genitals and the front surface of the thigh. Sometimes the pain can be one-sided, but over time it still appears on the opposite side.
The main feature of the disease is the gradual manifestation of symptoms and an increase in their intensity. In the later stages, patients complain of a decrease in the amount of urine, the appearance of acute lower back pain, persistent urinary tract infections that develop into chronic renal failure.
Diagnostics
Ormond's disease is characterized by a number of laboratory and instrumental symptoms that help to distinguish it from other diseases of this group.
In a clinical blood test, you can see an acceleration of the sedimentation time of red blood cells, a biochemical analysis indicates an increased amount of alpha globulins and C-reactive protein, and an increase in the content of urea and creatinine (signs of chronic renal failure).
Of instrumental studies, radiography is performed to see the contours of the kidneys and muscles near them. Then a contrast agent is injected into the blood and it is observed how quickly it is filtered out. In this case, you can see the contours of the cups and pelvis of the kidneys, the shape, location of the ureters and their deviation to the central plane of the body. In addition, scintigraphy can be performed with labeled radioactive atoms in order to assess the functional state of the urinary system.
Do not forget about such a method as ultrasound. He visualizes not only hollow structures, but also blood flow in important vessels of the retroperitoneal space. Sometimes, as an additional method, CT diagnostics of retroperitoneal fibrosis is used to clarify the topography of individual organs before surgery. If necessary, you can resort to wreath printing. This method allows you to visualize the inferior vena cava, its branches and collaterals, see their location and patency throughout the vessel.
It is important not only targeted diagnostics, but also the search for the etiological factor that gave impetus to the development of the disease. Therefore, it is recommended to examine the pelvic organs and abdominal cavity for hidden inflammatory diseases.
Differential diagnosis
Clinical manifestations of retroperitoneal fibrosis (Ormond's disease or RPF) must be distinguished from other pathological narrowings of the ureters (strictures and achalasias), as well as from bilateral hydronephrosis. The latter is rare, since a violation of the outflow of urine from two sides at once will lead to acute renal failure and require emergency treatment, in contrast to slowly progressing fibrosis.
To clarify the diagnosis and differentiate it from oncological pathology, several puncture biopsies can be done with subsequent histological examination. In rare cases, it is possible to establish the fact of the disease only after diagnostic laparoscopy with the collection of material for pathological examination.
In addition, an important difference between Ormond's disease is that the clamping of the ureters occurs at the level of their intersection with the iliac arteries, and not in an arbitrary place. Sometimes there is a need to distinguish RPF from atypically located pancreatic cysts, tumors of the digestive tract, tuberculosis of the kidneys and ureters.
In addition to all of the above, the patient may need a consultation with a pulmonologist, TB specialist, oncologist and cardiologist.
Treatment
What is the doctor’s tactic for patients diagnosed with retroperitoneal fibrosis? Treatment for the most part depends on the cause of the disease. If this is a secondary RPF, then in addition to symptomatic therapy, they try to eliminate the underlying disease - to discontinue medication, sanitize the focus of a chronic infection, or operate on a tumor. With an unknown etiology of the disease, steroid drugs or immunosuppressants are prescribed that inhibit the growth of new tissues.
Palliative therapy comes down to maintaining the patency of the ureters in all departments and to avoid possible complications.
Where to treat Ormond's disease? It all depends on the stage of the disease. Initially, you can arrange for medication at home or in the outpatient clinic, as there are no serious morphological changes. In advanced cases, the patient needs to be in a urological hospital, sometimes even in intensive care.
Conservative therapy is appropriate only if the ureters are passable enough and kidney function is not impaired to a large extent. To slow down and stop the fibrosis process, use:
- glucocorticosteroids ("prednisone");
- non-steroidal anti-inflammatory drugs ("Ibuprofen", "Paracetamol", "Celecoxib");
- immunosuppressants (Azathioprine, Metronidazole);
- absorbable preparations, enzymes (hyaluronidase, aloe juice).
The following scheme is considered the most effective:
- 25 mg of Prednisolone in the first month, with a gradual dose reduction in the next 3 months;
- daily 20 mg of Esomeprazole at bedtime;
- a half-year course of "Wobenzym" at 15 tablets a day;
- one month after the start of taking Prednisolone, add Celecoxib in a dosage of 100 mg.
If necessary, in addition to the pathogenetic, symptomatic therapy is also prescribed. Against the background of taking the drugs, the condition of the patients improves. Unfortunately, this disease is characterized by a relapse. Therefore, after drug withdrawal in most patients, the symptoms return and become even stronger.
Surgical treatment is prescribed only in case of complications, such as:
- pronounced expansion of the ureters ;
- gross deformation of retroperitoneal structures;
- arterial hypertension associated with renal failure, and others.
Surgeons perform ureteroplasty, establish nephrostomy, and return patency of the inferior vena cava and its tributaries.
Power Features
Retroperitoneal fibrosis is a disease that requires a strict diet. Patients need to exclude fried, salty, spicy and smoked foods from the diet. It is especially important to monitor your diet in the early stages of the development of the disease, as complications are easier to prevent than to treat later. In addition, diet helps slow down the development of Ormond syndrome.
If, at the first visit to the hospital, a narrowing of the ureter is already present, it is recommended that he drink as much water as possible. This is necessary not only to maintain the tone of the urinary system, but also to eliminate the metabolites that accumulate in the blood due to decreased kidney function.
The establishment of a nephrostomy for the excretion of urine through a catheter can quite strongly affect the patient's daily life. For drainage, special care is needed to prevent maceration and inflammation of the tissues around the tube.
Complications
J. Ormond's disease, like other systemic disorders, has its complications. The most dangerous of these is the cessation of outflow of urine or anuria. This leads to rapid intoxication and the development of chronic renal failure, as urine stagnates in the pyelocaliceal system and damages kidney cells.
In second place in terms of occurrence is hypertension. It appears due to a gradual decrease in the diameter of the renal artery and, as a result, blood flow in it. This leads to a compensatory increase in renin concentration and an increase in systemic pressure.
The third complication is varicose veins with compression of the inferior vena cava and impaired outflow from the lower extremities. In advanced cases, poorly healing ulcers may appear.
If intestinal loops are involved in the process of tissue fibrosis, intestinal obstruction occurs. It is manifested by stool retention, bloating and intoxication.
Prevention
As with many other diseases, the main method of preventing the appearance of retroperitoneal fibrosis is the elimination of foci of chronic infection, competent and timely treatment of hepatitis, tuberculosis or systemic vasculitis.
In addition, doctors recommend that you carefully monitor your health, especially if the family already has established cases of collagenopathy. This will allow you to identify the disease at an early stage and timely start treatment before the development of complications, which in the future will contribute to improving the quality of life and its prolongation.
Forecast
The development of Ormond disease in each individual patient depends on the stage in which the pathology was detected, and on the rate of progression of fibrosis. It is also important to consider the state of the urinary system, the presence of complications and birth defects. In most cases, conservative therapy brings a temporary positive effect. The most successful was the surgical treatment method, which consists in the plastic surgery of the ureter and its movement. After the operation, it is recommended to take steroid preparations for a long time to improve the prognosis of survival and quality of life. Relapses are possible, but they are significantly delayed in time compared with isolated drug therapy.
The main cause of death is chronic renal failure. Therefore, the forecast remains unfavorable. In some cases, when the disease was detected at a late stage, the probability of death is more than sixty percent. Therefore, the sooner a pathology is detected, the more successful the treatment will be.