Overview of retroperitoneal fibrosis
Retroperitoneal fibrosis, also known as idiopathic retroperitoneal fibrosis, is a chronic nonspecific inflammation of the fascia and adipose tissue of the retroperitoneum that gradually evolves into a fibroproliferative disease, and the lesions may progress to the abdominal cavity, pelvis and other tissues. It causes obstruction by compression of the retroperitoneal cavity organs.
Etiology
The etiology of this disease is unknown and may be related to a nonspecific response of the body to some chronic infection and irritation foci. It may also be related to the autoimmune response, genetic factors, etc.
Symptoms
It can develop at any age and even in newborns, but is more common in middle-aged people. Male patients are twice as common as females. The disease usually starts insidiously and has a long course. Early manifestations include dull pain in the lower abdomen on both sides, mostly in the lateral lower abdomen, lumbosacral region or lower abdomen. Other symptoms include anorexia, lethargy and fatigue, one or both legs, scrotal swelling or moderate fever, and occasional palpable masses in the abdomen or pelvis. In the progressive stage, the clinical manifestations are often the pressure or involvement of neighboring organs, such as causing ureteral stenosis can cause proximal infection or dilatation, which can produce lumbar or cribriform angle pain, urinary frequency and nocturia; bilateral ureteral compression is suddenly anuria; due to the presence of pyelonephrosis or renal infections, the lumbar tenderness is very common. Hypertension is common (one of the causes of headache), mostly due to renal obstruction. Gastrointestinal symptoms can be related to uremia or direct damage to the gastrointestinal tract (e.g., displaced stenosis). Biliary and pancreatic duct stenosis, if involving the portal or splenic veins, has been reported to cause portal hypertension with esophagogastric fundal varices and peritoneal effusion. Compression or obstruction of the lymph, veins or small arteries in the retroperitoneal cavity may result in one or both legs swelling, penile swelling or scrotal edema, and even abdominal wall vein filling or varicose veins, thrombosis of the lower extremities, and weak pulses in the ends of the lower extremities and intermittent claudication. It may be accompanied by fibrosis in other areas (e.g., mediastinal bile ducts, etc.), or even sclerosing cholangitis, Peyronie’s disease (Peyronie’s disease, in which the cavernous body of the penis is sclerotized and produces fibrotic, painful erections, i.e., fibrosing cavernous body inflammation), and so on.
Examination
1. Laboratory examination
There is often increased blood sedimentation, varying degrees of anemia and increased white blood cell count, occasionally eosinophilia, protein electrophoresis α and λ globulin increase. Urine routine examination can be normal or have a small number of white blood cells, red blood cells, and later can have uremia. Therefore, patients with uremia should pay attention to whether the urine is normal due to retroperitoneal fibrosis.
2.X-ray examination
X-ray urography can be seen on one side or both sides of the ureter displacement, diagnostic significance of the manifestation of the ureter in the middle of the gradual thinning with segmental stenosis, which is different from the tumor or stone-induced stenosis, the latter does not gradually thinning but only irregular narrowing. Involvement of the gastrointestinal tract can be seen on double contrast X-ray with segmental stenosis of the affected bowel, such as the duodenum, and pelvic fibrosis can lead to stenosis and straightening of the rectum with elevation of the bladder in the form of a teardrop. Venography can show stenosis of the inferior vena cava or iliac veins.
3. CT and MRI
Fibrous plaques or abnormal soft tissue masses can be found in most cases, and enhancement scans show dense fibrous tissue signs. As fibrosis develops laterally, the angle between the aorta and the left psoas major muscle and between the inferior vena cava and the right psoas major muscle changes, and also shows proximal ureteral dilatation. Since magnetic resonance is not superior to CT in the display of fibrous plaques, but it can show changes in vascular flow velocity, CT is preferred, and magnetic resonance is chosen when further hemodynamic changes are needed.
4. B-mode ultrasonography
PRF mass is hypoechoic or anechoic, no characteristic performance. The degree of urinary tract obstruction and hydronephrosis can also be observed.
Diagnosis
The diagnosis of this disease focuses on strengthening the understanding of this disease and raising vigilance. Anyone who regularly takes painkillers, methylergometrine, etc., has unexplained back pain, abdominal pain, lumbosacral or lower abdominal pain, portal hypertension, ascites, protein-losing enteropathy, etc. B-mode ultrasonography, CT and X-ray examination may help in the diagnosis.
Differential diagnosis
It should be distinguished from retroperitoneal lymphoma, proliferative lymphadenitis, metastatic tumor, primary tumor, periaortic hematoma and amyloidosis. In addition to clinical manifestations it is mainly dependent on imaging.
Treatment
1. Glucocorticoid
Early application of glucocorticosteroids can be effective within a few weeks, and may even cause the mass to shrink or disappear significantly. For patients with mild to moderate urinary tract lesions, old and frail or systemic diseases, prednisone drugs are more appropriate. Sometimes they are also used for preoperative preparation or postoperative prevention of recurrence. The initial dose of 30-60mg of prednisone or prednisolone per day, to be stabilized after the dose is gradually reduced to the lowest effective maintenance dose, at least 3 months. Some people use hormone and azathioprine to achieve better results, radiotherapy efficacy is not yet certain.
2. Surgery
Although fibrosis itself rarely requires surgical excision, hormonal therapy is rarely useful once a large amount of fibrosis has developed. Surgery is required when organ compression affects function.
A single bilateral ureteral release, in which the ureters can be wrapped in greater omentum and moved laterally, may provide better and more sustained relief. There is a high recurrence rate with laxation alone.
In advanced stages, for severe urinary tract obstruction, percutaneous nephrostomy and drainage, which is superior to retrograde ureteral intubation or bracing, not only provides timely relief of symptoms, but also allows monitoring of renal function by urinary electrolyte measurements, which avoids the need for hemodialysis in most patients.
Questions you may be concerned about
What about retroperitoneal fibrosis caused by arteritis?
Post-peritoneal fibrosis caused by arteritis can be treated with anti-inflammatory drugs such as prednisolone, tamoxifen, azathioprine, etc. under the guidance of physicians, and surgical treatment if necessary.
1. Drug therapy: such as glucocorticoids, immunosuppressants, anti-inflammatory and antifibrotic drugs.
(1) Glucocorticosteroids: for example, prednisolone, etc., with anti-inflammatory and promote the maturation of fibrotic tissues; especially for active inflammation in the middle and early retroperitoneal fibrosis patients; pregnant women and breastfeeding women should try to avoid using. The use of glucocorticoids may lead to water, salt, sugar, protein, fat metabolism disorders, weakened immunity and other adverse reactions.
(2) Immunosuppressants: such as methotrexate and azathioprine. By suppressing excessive immune response and tissue damage, they can make hormones play a better therapeutic effect; after using them, they may lead to headache, insomnia, sensory retardation and other adverse reactions.
(3) Anti-inflammatory and antifibrotic drugs: such as tamoxifen, which can inhibit lipoprotein oxidation; the use of the drug may lead to nausea and vomiting, loss of appetite and other gastrointestinal side effects.
2. Surgery: When patients have symptoms of ureteral obstruction, surgical treatment usually needs to be considered. Such as ureteral stricture resection plus end-to-end anastomosis, laparoscopic ureteral release.
Patients with retroperitoneal fibrosis caused by arteritis should go to the general surgery department of the hospital in time and follow the doctor’s instructions for treatment, so as not to affect normal life.
Prognosis
Although the disease is progressive, sometimes it can be relieved by itself, and the prognosis is generally good. However, when serious complications such as renal insufficiency occur in the late stage, it is more difficult to treat.
Questions you may be concerned about
How many years can you live with retroperitoneal fibrosis?
Retroperitoneal fibrosis is characterized by proliferation of retroperitoneal fibrous tissue, which leads to extensive retroperitoneal fibrosis, and its clinical manifestations are closely related to the degree of compression of retroperitoneal tissues or organs (such as the ureter).
The disease is a somewhat self-limiting and slowly progressive disease with a good prognosis. If diagnosed in time and the obstruction is lifted, the disease can be relieved for a long time. If it is not treated in time and effectively, it can lead to death due to complications such as renal insufficiency.
Retroperitoneal fibrosis is extremely rare, and the cause is still unclear. It may be related to abnormalities in the immune system, smoking, exposure to asbestos, and the effects of certain drugs.
Retroperitoneal fibrosis can be improved through “early detection, early diagnosis, early treatment” to improve the prognosis of the disease, reduce the adverse effects of the disease, patients can work and live as normal after treatment. It is recommended that patients actively carry out regular treatment and avoid self-medication. You should have a regular routine, eat a healthy diet, and avoid spicy and stimulating foods.
How long does it take to cure retroperitoneal fibrosis?
Retroperitoneal fibrosis usually takes about three months to treat, and the specific treatment time should be determined according to the individual’s physical condition and the severity of the disease.
Retroperitoneal fibrosis is a serious, autoimmune disease caused by fibroproliferative disease, the lesions may develop into the pelvis, abdominal cavity and other tissues, may cause pressure on the retroperitoneal cavity organs and obstruction, may appear weakness, nausea, vomiting, lower limb edema and other symptoms.
You can take azathioprine, triamcinolone acetonide and other drugs under the guidance of the doctor for treatment, usually about a month can slowly recover, and gradually reduce the dosage at a later stage, at least need about three months. In addition, it is usually recommended to eat more high-protein high-fiber food, pay attention to a balanced diet, do not eat spicy stimulation greasy food, eat more fresh fruits and vegetables, to maintain a smooth bowel movement.
Appearance of retroperitoneal fibrosis, need to go to the hospital in time.