The term retroperitoneal fibrosis (also known as retroperitoneal fibrosis, Ormond syndrome or Morbus Ormond) is used by physicians to describe an increase in connective tissue that occurs between the spine and the posterior peritoneum. The nerves, ureters and vessels are mainly “walled up”.
What is retroperitoneal fibrosis?
Retroperitoneal fibrosis is a relatively rare disease (1 in 200,000), but it predominantly affects men. The first signs of retroperitoneal fibrosis appear in middle age. Retroperitoneal fibrosis is further divided into two forms: the idiopathic (primary) and secondary form (Ormond syndrome). In the primary form, also known as Ormond’s disease or Albarran-Ormond syndrome, there is no triggering event. For about menorrhagia (long and heavy menstrual bleeding), please visit bittranslators.com.
Sometimes doctors assume that an autoimmunological process was the cause of the development. In the secondary form (also known as Ormond syndrome), retroperitoneal fibrosis is sometimes triggered by primary biliary cirrhosis, Crohn’s disease or Sjögren’s syndrome, Erdheim-Chester disease or also due to granulomatosis or polyangiitis.
Joaquin Albarran, a Cuban urologist, was the first physician to describe the disease in 1905. A more extensive description and documentation by John Kelso Ormond, a urologist from the USA, followed in 1948.
Causes
Even if secondary forms of the disease can sometimes trigger retroperitoneal fibrosis, the actual cause is still unknown. This applies to both forms of retroperitoneal fibrosis.
Symptoms, Ailments & Signs
The patient usually complains of dull pain that is difficult to localize but not comparable to colic, which is mainly reported in the scrotum, the flanks or the back. In almost all cases the ureters become walled; this causes urine to back up in the kidneys, creating the possibility of hydronephrosis.
Occasionally, however, larger arteries, the aorta, peripheral nerves or even the intestinal tract itself, the pancreas and biliary system and pelvic organs can be affected. Another sign of retroperitoneal fibrosis is swollen legs ; This is because there are obstructions to drainage in the lymph vessels and veins. In a few cases, inflammatory or fibrotic changes in the pleura, pericardium, paranasal sinuses, orbits, thyroid gland or mediastinum occur.
Diagnosis & course of disease
A histology (examination of the fine tissue) is carried out so that the retroperitoneal fibrosis can be detected. Occasionally, however, imaging procedures can already provide clear indications that retroperitoneal fibrosis is present. The confirmation of diagnosis, which only exists on the basis of imaging procedures, is permitted if a tissue sample would entail too high a risk.
In many cases, the examination using magnetic resonance imaging or computer tomography already shows a significant change in the connective tissue around the aorta (significant increase). Sometimes renal arteries or directly adjacent structures can also be affected.
However, if the doctor recognizes atypical characteristics (displacement symptoms, enlargement of the lymph nodes, atypical localization), a tissue sample must be taken. This is so that any granulomatous or malignant processes can be ruled out.
Patients usually respond well to immunosuppressive therapy. However, since there is no actual therapy recommendation to date, each case must be individually observed, analyzed and subsequently treated. However, once healed, there is a chance that retroperitoneal fibrosis will recur.
Kidney damage may occur during retroperitoneal fibrosis due to urinary retention. Mortality (mortality) is between 10 percent and 20 percent; According to the latest findings and studies, however, the mortality rate is already below 10 percent, so that nowadays a significantly better chance of recovery must be assumed.
Complications
Retroperitoneal fibrosis is a serious autoimmune disease that can be accompanied by several complications. Most complications result from compression of organs resulting from increased fibrous connective tissue regeneration. Immunosuppressive therapy usually responds very well. However, if this is not carried out long enough, a relapse often occurs.
However, complications can only be prevented with the help of such therapy. Compression often affects the kidneys, ureters, abdominal aorta, common iliac arteries, and inferior vena cava. The compression of the ureters can lead to a backlog of urine, which damages the kidneys in the long term. This often results in urinary tract infections and inflammation of the renal pelvis, which can only be treated with antibiotics. In the long term, urinary retention leads to severe kidney damage, which in very severe cases even necessitates a kidney transplant.
The intestines are also often affected. The increased growth of the connective tissue can encourage it to become narrowed. There is a risk of developing an intestinal obstruction, which must be treated immediately with surgery to prevent large parts of the intestine from dying off. Furthermore, the compression of large veins and the associated blood flow disorders can lead to thrombosis, which can only be broken down again with the help of blood-thinning medication.
When should you go to the doctor?
Retroperitoneal fibrosis should always be treated by a doctor. This disease does not heal itself and in most cases the symptoms worsen significantly. Retroperitoneal fibrosis cannot be treated by self-help either, so a medical examination is always necessary.
The doctor should be consulted for retroperitoneal fibrosis if the patient suffers from severe pain in the scrotum. The pain can also occur on the back or flanks and significantly reduce the quality of life of those affected. Furthermore, swollen legs of the patient can indicate retroperitoneal fibrosis, the swelling should be examined by a doctor if it occurs over a longer period of time and does not go away on its own.
In most cases, retroperitoneal fibrosis can be diagnosed and treated by a general practitioner or urologist.
Treatment & Therapy
So far, there are no controlled studies on any treatments or therapeutic approaches. This is because retroperitoneal fibrosis is relatively rare. Due to the rarity, the recommendations are mainly based on the results of smaller case series (casuistics); sometimes the first spontaneous cures (spontaneous remissions) have already been documented.
However, these spontaneous remissions are the absolute exception. As a rule, the duration of treatment is between 12 and 24 months; a recurrence of retroperitoneal fibrosis cannot be ruled out. If there is a problem with the transport of urine, the doctor must restore the outflow. As part of an operation, an internal splint (with catheter) is applied to the patient. The ureter may also need to be exposed. In rare cases, such as when a chronic infection has been diagnosed, a kidney may need to be removed (nephrectomy).
Since there are no actual recommendations as yet on how retroperitoneal fibrosis should be treated, there are also no uniform plans as to which medications are recommended. However, physicians have found that immunosuppressants (azathioprine or corticosteroids) or tamoxifen are particularly promising. Methotrexate, cyclophosphamide, cyclosporin A, mycophenolate mofetil and colchicine have also been used successfully.
Occasionally, however, special treatments may also be required if complications occasionally occur. As the bowel becomes constricted, bowel obstruction is possible. The intestinal obstruction must be treated surgically. If there is a problem with the outflow of urine, a urinary tract infection can sometimes occur.
The infection can progress to the renal pelvis (pelvic inflammatory disease), requiring the patient to be treated with antibiotics. If large veins are narrowed, this can lead to thrombosis or impairment of blood flow. In those cases, anticoagulants are administered.
Prevention
Since no causes are known to date as to why retroperitoneal fibrosis develops, there are also no preventive measures. However, anyone suffering from diseases that could be associated with retroperitoneal fibrosis should consult a doctor at the first sign to clarify whether or not retroperitoneal fibrosis is present.
Aftercare
In most cases, those affected with retroperitoneal fibrosis have only limited follow-up measures available. The person concerned should first and foremost see a doctor very early on, so that there are no further complications or other complaints for the person concerned. The sooner a doctor is consulted, the more promising is usually the further course of the disease.
Therefore, the affected person should contact a doctor at the first sign of the disease. Most patients with this disease are dependent on a surgical procedure, which can relieve the symptoms permanently. After such a procedure, the person concerned should definitely rest and take care of his body. Efforts or physical and stressful activities should be avoided in order not to unnecessarily burden the body.
Physiotherapy or physiotherapy measures may also be necessary, whereby the person concerned can carry out some of the exercises at home in order to speed up the treatment. Even after a successful procedure, regular check-ups and examinations by a doctor are very important in order to monitor the current condition of the retroperitoneal fibrosis. As a rule, this disease does not reduce the patient’s life expectancy.
You can do that yourself
In order to improve their quality of life, those affected must absolutely adhere to the cortisone dose prescribed by the doctor. In the course of the treatment, the body adapts to the dose supplied to it and reacts by reducing or completely stopping the body’s own production of cortisone. However, the stress hormone cortisone is essential for the body’s survival, as it is involved in many metabolic processes. Sudden discontinuation or a significantly reduced dose can lead to threatening metabolic disorders, a severe relapse or even secondary diseases.
All side effects and abnormalities should be noted by the person concerned and passed on to the doctor as part of the regular check-ups. This can react accordingly with an adjusted dose of medication or other prescribed therapeutic agents.
Some of those affected were able to achieve steady improvement through a targeted change in diet to a balanced and healthy diet. Such an adjustment of the nutrition plan can be optimally carried out with the help of a nutritionist and adapted to individual needs. As a result, the immune system is strengthened, excess weight is reduced and the body is supplied with all the minerals and vitamins it needs for a well-functioning metabolism.
Contact with self-help groups can eliminate uncertainty in dealing with the disease. In conversations you get a lot of empirical values, doctor’s recommendations and helpful tips for everyday life and possible further therapies.
