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 structures located there (lymphatic and blood vessels, ureters).
Retroperitoneal fibrosis (another name for the disease) is a type of systemic idiopathic fibrosis, which also includes mediastinal fibrosis, Riedel's struma, sclerosing cholangitis, Peyronie's disease and others. All of the above are local manifestations of the same disease.
Prevalence
Ormond's disease is, fortunately, extremely rare: only one case in two hundred thousand people. As a rule, it is detected in men from thirty to sixty years. Women of the same age group get sick twice as often. Mortality is low, and in most of the cases described, it was caused by complications, and not by the disease itself.
The ureters, which are well covered with subcutaneous fat, are most often affected by compression. They find themselves, as it were, in a dense narrowcase and, due to its rigidity, cease to fulfill its function.
Classification
Clinicians differentiate between primary and secondary Ormond's disease. Primary, or idiopathic fibrosis, occurs on its own, for unknown reasons. Scholars have several explanations for 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 disease. It can be a chronic infection (or carriage of a pathogen), connective tissue diseases, and others.
Reasons
As mentioned earlier, the causes of Ormond's illness are not known for certain. There are several theories trying to explain the changes that occur in the body.
- Inflammatory. As a result of prolonged local tissue edema, impregnation with fibrin and other acute phase proteins, they become rigid and inactive.
- Immune. Due to a malfunction of the protective systems, the body begins to produce antibodies against its own tissues, injures them and, in response to damage, replaces these areas with fibrin.
- Gennaya. Breakage in the region of the gene responsible for the structure of connective tissue. Manifested in a change in the structure of retroperitoneal tissue.
A number of researchers suggest that retroperitoneal fibrosis can be attributed to a number of truecollagenosis. There are certain factors that contribute to the manifestation of the disease. These include cancer, 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 stop migraine attacks can also contribute to the formation of fibrosis. But in most cases, the trigger factor cannot be determined.
Pathogenesis
Most of the retroperitoneal fibrosis (Ormond's disease) begins with the appearance of compacted areas at the level of the fourth or fifth lumbar vertebrae. The iliac arteries and ureters are located there.
Over time, the affected area expands and, capturing more and more fiber, descends to the cape of the sacrum, and also to the sides to the gates of the kidneys. In almost half of the cases, this process is two-way. Rough 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 deteriorates over time.
Impaired patency of the ureters leads to stagnation of fluid in the kidneys, hydronephrosis, chronic inflammation and, as a consequence, chronic renal failure. In rare cases, this is accompanied by intestinal obstruction or obstruction of large vessels.
Symptoms
There are no signs that would unambiguously characterize Ormond's disease. It all depends on the stage of the process, its prevalence, activity and specific features of the human body. The duration of the latent course of the disease can vary from two months to eleven years.
All the symptoms of Ormond's disease can be divided into three periods, based on the time of their appearance:
- beginning of illness;
- increased fibrous fibers;
- "tightening" of fibrin and compression.
A 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, groin, genitals and front 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 the increase in their intensity. In the later stages, patients complain of a decrease in the amount of urine, the appearance of acute pain in the lower back, persistent urinary tract infections that develop into chronic kidney failure.
Diagnosis
Ormond's disease is characterized by a number of laboratory and instrumental features that help distinguish it from other diseases of this group.
In a clinical blood test, you can see the acceleration of the time of sedimentation of red blood cells, biochemical analysisindicates an increased amount of alpha-globulins and C-reactive protein, an increase in the content of urea and creatinine (signs of CRF).
From instrumental studies, X-rays are taken to see the contours of the kidneys and muscles around 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, radiolabeled scintigraphy can be performed to assess the functional state of the urinary system.
Do not forget about such a method as ultrasound. It visualizes not only hollow structures, but also the blood flow in the 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 venokavagrafiya. 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 diagnosis, but also the search for the etiological factor that gave impetus to the development of the disease. Therefore, it is recommended to conduct a pelvic and abdominal examination for hidden inflammatory diseases.
Differential Diagnosis
Retroperitoneal fibrosis (Ormond's disease or RPF) must be distinguished from others by clinical manifestationspathological narrowing of the ureters (strictures and achalasia), as well as from bilateral hydronephrosis. The latter is rare, as obstruction of the outflow of urine from both sides at once will lead to acute renal failure and require emergency treatment, unlike slowly progressive fibrosis.
To clarify the diagnosis and differentiate it from oncological pathology, several puncture biopsies can be made followed by a 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 post-mortem examination.
In addition, an important difference between Ormond's disease is that clamping of the ureters occurs at the level of their intersection with the iliac arteries, and not in an arbitrary place. Sometimes it becomes necessary to distinguish RPF from atypically located pancreatic cysts, neoplasms of the digestive tract, tuberculosis of the kidneys and ureters.
In addition to all of the above, the patient may need to consult a pulmonologist, phthisiatrician, oncologist and cardiologist.
Treatment
What is the doctor's tactics for patients diagnosed with retroperitoneal fibrosis? Treatment largely 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 - cancel the medication, sanitize the focus of a chronic infection, or operate on the tumor. When the etiology of the disease is not clear, steroid drugs are prescribed.or immunosuppressive drugs that inhibit the growth of new tissues.
Palliative care is about maintaining the patency of the ureters in all departments and avoiding possible complications.
Where to treat Ormond's disease? It all depends on the stage of the disease. Initially, you can organize medication at home or in an outpatient clinic, since 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 sufficiently patent and renal function is not significantly impaired. To slow down and stop the process of fibrosis use:
- glucocorticosteroids ("Prednisolone");
- 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:
- 25mg "Prednisolone" in the first month, with a gradual decrease in the dose over the next 3 months;
- Esomeprazole 20mg daily at bedtime;
- semi-annual course "Wobenzym" 15 tablets a day;
- one month after starting Prednisolone, add Celecoxib at a dosage of 100 mg.
If necessary, in addition to pathogenetic, symptomatic therapy is also prescribed. While taking drugsthe condition of the patients is improving. Unfortunately, this disease is characterized by a recurrent course. Therefore, after discontinuation of drugs in most patients, the symptoms return and become even worse.
Surgical treatment is prescribed only in case of complications such as:
- pronounced dilatation of the ureters;
- rough deformity of retroperitoneal structures;
- arterial hypertension associated with renal failure, and others.
Surgeons perform ureteroplasty, install nephrostomy, restore the patency of the inferior vena cava and its tributaries.
Features of food
Retroperitoneal fibrosis is a disease that requires a strict diet. Patients should exclude fried, s alty, 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, since complications are easier to prevent than to treat later. In addition, the diet helps slow the development of Ormond's syndrome.
If, at the first time a person goes to the hospital, narrowing of the ureter is already present, then he is advised to drink as much water as possible. This is necessary not only to maintain the tone of the urinary system, but also to remove metabolites that accumulate in the blood due to a decrease in kidney function.
The daily life of a patient can be quite affected by the installation of a nephrostomy to remove urine through a catheter. Drainage requires special care 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 them is the cessation of the outflow of urine or anuria. This leads to rapid intoxication and the development of chronic renal failure, as urine stagnates in the pyelocaliceal apparatus and damages kidney cells.
Arterial hypertension is in second place in terms of occurrence. It appears due to a gradual decrease in the diameter of the renal artery and, as a result, the 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 due to 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 occurrence 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 he alth, especially if there are already established cases of collagenopathy in the family. This will allow to identify the disease at an early stage and start treatment in a timely manner before the development of complications, which in the future will improve the quality of life and prolong it.
Forecast
The development of Ormond's disease in each individual patient depends on the stage at which the pathology was detected, and on the rate of progression of fibrosis. It is also important to take into account 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 recognized as the method of surgical treatment, which consists in the plasticity of the ureter and its movement. After surgery, it is recommended to take long-term steroid drugs to improve the prognosis of survival and quality of life. Relapses are possible, but they are significantly delayed in time compared to drug therapy alone.
The main cause of death is chronic kidney failure. Therefore, the prognosis 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 the pathology is detected, the more successful the treatment will be.
