Retroperitoneal fibrosis

Introduction Idiopathic retroperitoneal fibrosis refers to the inflammatory reaction and fibrosis of the retroperitoneal connective tissue caused by different causes, forming dense fibrous tissue surrounding and compressing the organs behind the peritoneum (such as ureter and adjacent large Blood vessels). Compression of the ureter can cause upper urinary tract obstruction, which can affect kidney function and lead to uremia. The pathological feature is centered on the lower part of the abdominal aorta, with a dense band of fibrous tissue extending around the common iliac vessels and extending into the inferior vena cava.

Cause

Causes

The cause of this disease is unknown and may be related to the following factors:

1. Allergic theory: RPF is often accompanied by abdominal aortic aneurysm-like dilation, severe aortic wall calcification, ureteral obstruction and peri-aortic inflammation. Recently, Bullock suggested that RPF is an allergic reaction caused by leakage of insoluble inflammatory fat from the arterial wall thinning from the atheromatous plaque, so it should be renamed as "chronic aortic inflammation". An insoluble polymer of oxidized lipids and proteins is sometimes found in macrophages and lymph nodes in the atherosclerotic blood vessels and in atherosclerotic plaques. Immunohistochemical studies have shown that the substance contains IgG, and a small amount of IgM. This change may be the result of some kind of autoimmune response, especially when it is effective against steroid therapy.

2. Ergot compound theory: Graham reported that RPF occurred in 2 of the patients treated with ergometrine. Later, a group of 27 patients were treated with ergometrine for headache and RPF, and the drug was discontinued. Some cases returned to normal. The above phenomenon suggests that ergometrine has a causal relationship with RPF. On the contrary, Blandy et al said that the RPF patients reported in the UK have not taken this drug or any other ergot compound. A ergometrine is a serotonin blocker that increases endogenous serotonin levels by competitive inhibition of the receptor site. Graham suggested that serotonin can cause a carcinoid syndrome-like abnormal fibrotic response in susceptible patients. Bromocriptine is a derivative of ergot alkaloids, but it is not a serotonin blocker and may be associated with retroperitoneal and mediastinal fibrosis. It is possible that ergot alkaloids cause an allergic or autoimmune response as a hapten, but so far there is no satisfactory evidence.

3. Other causes: Some people have suggested that RPF is associated with taking painkillers; some are suspected of beta-adrenergic blockers, but Pryor believes that this drug may have been used to treat hypertension caused by RPF. Not the cause of the disease. The pathological feature is centered on the lower part of the abdominal aorta, with a dense band of fibrous tissue extending around the common iliac vessels and extending into the inferior vena cava. The upper edge is usually below the renal artery, but fibrosis can occur around the thoracic aorta. Expressed as a flat, solid gray-white fibrous plaque. The dividing line is usually clear without an envelope, and when the lesion expands, the structure of the retroperitoneal space is surrounded, but does not invade the walls of these structures. Typically, the bilateral bilateral ureters are surrounded.

an examination

symptom

The symptoms of this disease are closely related to the course of the disease. The early symptoms of RPF are insidious. Mainly manifested as non-specific back pain, abdominal pain and flank pain, persistent dull or dull pain, can occur in any age or even newborn, but more common in middle-aged people. Male patients are twice as many as females, and both Caucasians and blacks can get sick. Usually the onset is concealed, the course of disease is longer, and the diagnosis is often made months or even years after some vague symptoms appear. The most common pain is usually the earliest symptom, and it is often blunt pain and discomfort in the lower abdomen, lumbosacral or lower abdomen. Other symptoms include anorexia, weight loss and fatigue. There may be swelling of one or both legs, swelling of the scrotum or moderate fever, and the abdomen or pelvic cavity may touch the mass. The clinical manifestations in the advanced stage are often symptoms of compression or involvement of adjacent organs. For example, ureteral stenosis can cause proximal infection or dilatation, can produce lumbar or rib horn pain, frequent urination and nocturia, bilateral ureteral compression. Sudden abdomen occurs; because of the often hydronephrosis or kidney infection, the tenderness of the waist is very common.

Sign

At the time of physical examination, there is often tenderness in the lower abdomen and lower back. The kidney area may have sneezing pain or touching the enlarged kidney. Retroperitoneal fibrous masses are generally not easily accessible. May be associated with high blood pressure.

Differential diagnosis

Differential diagnosis of posterior peritoneal fibrosis:

1. Patients with ureteral stones have lumbar pain and can radiate to the lower abdomen, vulva, and inner thigh. However, the pain caused by ureteral stones is mostly sudden, and the degree is more serious and unbearable. Routine urine tests before and after the onset may have red blood cells. Stone shadows can be found in the IVU.

2. Ureteral inflammation also shows low back pain, physical examination of the kidney area has sputum pain. However, there are often urinary tract irritation such as frequent urination, urgency, and dysuria. IVU can be seen with ureteral dilatation or stenosis, but no bilateral ureters are displaced to the center at the same time. B-ultrasound and CT examination have no space-occupying lesions around the ureter and blood vessels.

3. The inferior vena cava ureter can be manifested as right lower back pain, B-ultrasound and IVU can find right hydronephrosis, the right ureter is expanded and displaced to the midline, making the ureter an "S" shape, which is helpful for diagnosis.

4. The ureteral tumor is mainly characterized by dull pain in the waist. When the patient discharges the cord-like blood clot, it may be accompanied by renal colic, but in addition to the pain, the patient also has different degrees of hematuria. IVU can be seen in hydronephrosis, ureteral filling defects and cup-like changes or renal non-development. Cystoscopy sometimes shows tumors coming out of the ureteral orifice or ureteral orifice spurting. CT examination showed no space-occupying lesions around the ureter.

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