Retroperitoneal fibrosis

Introduction Retroperitoneal fibrosis (RPF) was first reported by French urology surgeon Albrran in 1905 and used the concept of retroperitoneal fibrosis until 2948, after Ormond reported 2 cases of retroperitoneal fibrosis, the case of this disease The report has gradually increased and is being recognized by more and more clinicians. The disease can occur at any age, but it is more common in people aged 40-60 years, accounting for about 2/3. The incidence of males is more common, which is 2 to 3 times that of females. Clinically, it is divided into the initial stage of the disease, the active stage and the stage of systolic phase of the fiber plate. Pain can be asymptomatic at first, and pain can occur later, mostly in the lower back or lower back and radiating to the lower abdomen, groin area, external genitalia or the anterior medial side of the thigh. Subacute inflammation manifests as abdominal pain, tenderness in the kidney, hypothermia, increased white blood cell count, increased erythrocyte sedimentation rate and fatigue, anorexia, nausea and vomiting, and weight loss.

Cause

A large number of clinical studies have found that the following factors may be associated with retroperitoneal fibrosis.

Self-immune deficiency

Clinical data show that 8% to 15% of patients with idiopathic retroperitoneal fibrosis can be accompanied by fibrosis other than retroperitoneal, with a history of scleroderma, eosinophilia, nodular arteritis, Systemic lupus erythematosus, glomerulonephritis, Riedle thyroiditis, sclerosing cholangitis, mediastinal fibrosis, and posterior fibrotic pseudotumor, suggesting that retroperitoneal fibrosis may be a local manifestation of systemic sclerosing disease. This indicates that the disease may be related to immunodeficiency.

2. Drug side effects

Similar cases have been reported in the literature since Grahacn reported that retroperitoneal fibrosis occurred in patients taking methyl methyryl amide (methysergide) in 1964. For long-term users, the incidence of retroperitoneal fibrosis can reach 10% to 12.4%. In addition, beta blockers (propranolol), antihypertensive drugs (methyldopa, reserpine, hydralazine), analgesics (aspirin, phenacetin) and other drugs are also available. The disease is induced, but their causal relationship with retroperitoneal fibrosis needs further study.

3. Infection and inflammation

As early as 1948, Ormond believed that the disease was a type of retroperitoneal inflammation, while Mathisen et al suggested that retroperitoneal fibrosis may be associated with infection of the abdominal and lower extremity viruses. The comprehensive literature reports that infection and inflammation associated with retroperitoneal fibrosis. There are: specific infections such as tuberculosis, syphilis, actinomycosis and various fungal infections; non-specific infections such as diverticulitis and appendicitis; ulcerative colitis, Crohn's disease, vasculitis of skin and subcutaneous tissue, etc. Inflammatory diseases; inflammatory lung injury, thrombophlebitis, pancreatitis and other inflammatory diseases can also cause retroperitoneal fibrosis. Retroperitoneal fibrosis induced by malignant tumors in malignant tumors accounts for 8% to 10% of all cases. Malignant tumors that cause connective tissue hyperplasia and fibrosis have been reported to include: breast cancer, lung cancer, thyroid cancer, gastric cancer, colon cancer, genitourinary cancer (kidney cancer, bladder cancer, prostate cancer, and uterine cancer), Hodgkin disease. And other malignant lymphomas, certain sarcomas, carcinoids, etc.

4. Aortic aneurysm

One of the more common types of retroperitoneal fibrosis reported in the literature is fibrosis around the aneurysm. Fibrosis can only surround the aneurysm, or it can grow laterally around the ureter and cause obstruction. The incidence of fibrosis around the aorta or aortic aneurysm is reported to be 5% to 23%. Injury clinical data show that trauma, retroperitoneal hematoma and radiation damage can lead to retroperitoneal fibrosis. Authors such as Asbestos Boulard and Sauni reported in 1995 and 1998 that 2 patients and 7 patients with retroperitoneal fibrosis had a history of close exposure to asbestos. The chest X-ray showed pleural plaque or calcified plaque and rounded lung. Incomplete expansion, etc., autopsy also found asbestos bodies behind the retroperitoneum. It is suggested that asbestos may be one of the causes of retroperitoneal fibrosis.

an examination

Blood routine

There may be a decrease in red blood cells and hemoglobin; an increase in eosinophils and a hematocrit of less than 33%.

2. Urine routine

One third of patients have proteinuria.

ESR

94% of patients had an increased erythrocyte sedimentation rate at the initial examination.

4. Renal function

Clinically, 75% of patients have varying degrees of renal dysfunction, manifested as oliguria and azotemia, such as elevated serum creatinine and urea nitrogen.

Alkaline phosphatase

In recent years, alkaline phosphatase is considered to be a marker of the disease, and elevated alkaline phosphatase is important for the diagnosis of this disease.

6. Histopathological examination

Fine needle aspiration (Fineneedleaspiration) biopsy can be performed under B-ultrasound and CT guidance. Biopsy can also be performed by laparoscopic examination of the mass of the tumor. In 1997, Stein first reported the use of fine needle aspiration under CT guidance to diagnose idiopathic retroperitoneal fibrosis, and 2/3 of the cases were diagnosed. The inflammatory cells such as fibroblasts and small lymphocytes are mainly aspirate, usually appearing separately, and a few appear at the same time. In addition, authors have reported that the X-ray guided transluminal vein biopsy of the peri-aortic mass was confirmed and followed up. For those who cannot be diagnosed, the laparotomy should be performed to take the pathological examination of the living tissue. On the one hand, the benign and malignant lesions can be diagnosed and diagnosed on the other hand. However, when taking biopsy specimens, attention should be paid to taking materials in a number of deeper parts to avoid misdiagnosis.

Differential diagnosis

1. Identification of diseases associated with susceptible organs or tissues

(1) ureteral disease: this disease and ureteral tumor, inflammatory stenosis, etc. can cause renal pelvis and ureteral water, should pay attention to the identification of the two.

(2) Pancreatic cancer: retroperitoneal fibrosis involves and causes occlusion of fat pad around the pancreas is often misdiagnosed as pancreatic cancer, and attention should be paid to their clinical and imaging characteristics.

(3) common bile duct disease or sclerosing cholangitis: the disease involving the common bile duct can cause jaundice and other manifestations, should pay attention to exclude biliary tract caused by diseases such as common bile duct disease or sclerosing cholangitis. However, retroperitoneal fibrosis can be part of systemic sclerosis with sclerosing cholangitis, and it is necessary to think of the possibility of both.

(4) knot, rectal disease: retroperitoneal fibrosis involving the knot, rectum, patients may have diarrhea, constipation, and even obstructive symptoms, easily misdiagnosed as nodules, rectal tumors or inflammatory diseases, and the two can cause each other, clinical Patients with diarrhea, constipation, and even obstruction should consider the possibility of retroperitoneal fibrosis after excluding the disease of the intestine itself.

(5) chronic intestinal obstruction and dysfunction of the small intestine: this disease involving the mesentery can cause chronic obstruction and motility disorders of the small intestine, often misdiagnosed as chronic incomplete obstruction or dysfunction of the small intestine. Therefore, when suffering from chronic incomplete obstruction or dysfunction of the small intestine, the disease should be considered.

2. Identification of certain primary diseases following the retroperitoneum

Such as retroperitoneal yellow granuloma, retroperitoneal hematoma, primary retroperitoneal tumors (including liposarcoma, fibrosarcoma, malignant lymphoma, fibroids, lymphangioma, leiomyomas, etc.) retroperitoneal amyloidosis. Identification of retroperitoneal malignant metastases In general, retroperitoneal malignant metastases can be identified on the outside of the ureter. Identification of aneurysms Aortic aneurysm or aortic aneurysm can form a fibrotic ring around the aneurysm or lateral growth and cause ureteral obstruction. Imaging showed that the surrounding aorta was tumor-like dilatation, while the aorta and common iliac artery involved in retroperitoneal fibrosis showed smooth, irregular stenosis changes.

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