Idiopathic retroperitoneal fibrosis

　　The etiology of idiopathic retroperitoneal fibrosis is unknown and may be related to the following factors:

　　1. Allergic theory

　　Idiopathic retroperitoneal fibrosis (RPF) often accompanies aneurysmal dilatation of the abdominal aorta, severe calcification of the aortic wall, ureteral obstruction, and periaortic inflammation. Sometimes, a non-soluble polymer of oxidized lipids and proteins can be found in macrophages and lymph nodes around atherosclerotic vessels and within atherosclerotic plaques. Immunohistochemical studies show that the substance contains IgG and a small amount of IgM. This change may be the result of some autoimmune reaction, especially as it is confirmed by the effectiveness of corticosteroid hormone treatment.

　　2. Ergot compound theory

　　Graham suggests that in susceptible patients, serotonin can cause an abnormal fibrotic reaction similar to that of carcinoid syndrome. 2-Bromoergocryptine is a derivative of ergot alkaloids but is not a serotonin antagonist and may be related to retroperitoneal and mediastinal fibrosis. It is possible that ergot alkaloids as haptens may cause allergic or autoimmune reactions, but there is still no satisfactory evidence to date.

　　3. Other causes

　　Some propose that RPF is related to the use of analgesics; some suspect β-adrenergic receptor blockers, but Pryor believes that it is more likely that this drug was used to treat hypertension caused by RPF, rather than the cause of the disease.

　　The main complications of idiopathic retroperitoneal fibrosis are ureteral obstruction, which can be accompanied by diseases such as sclerosing cholangitis, Reidel thyroiditis, Crohn's disease, arteritis, and other systemic diseases.

　　1. Obstructive uropathy at the renal pelvis-ureteral junction is a common disease of urinary tract obstruction that causes hydronephrosis. Due to the obstruction at the renal pelvis-ureteral junction, the smooth entry of urine from the renal pelvis into the ureter is obstructed, leading to the obstruction of renal pelvis emptying and the expansion of the renal collecting system.

　　2. Cholangitis sclerosans refers to a series of symptoms characterized by biliary inflammation and obstructive jaundice due to varying degrees of obstruction of the common bile duct or the bile duct caused by calculosis of the gallbladder neck or gallbladder duct, or compression or inflammation involving other benign diseases, leading to obstruction of the common bile duct or the bile duct.}}

　　3. Crohn's disease, also known as 'segmental enteritis', 'segmental colitis', 'chronic transmural colitis', etc., is characterized by an unknown etiology, more common in young people, and manifested as granulomatous inflammatory lesions, combined with fibrosis and ulcers, which can affect any part of the entire gastrointestinal tract, including the mouth and anus, with lesions distributed segmentally or saltatory, and can also affect tissues outside the intestines, especially the skin. The clinical manifestations are diverse due to the location, extent, and severity of the lesions, and can include abdominal pain, diarrhea, abdominal masses, fistula formation, and intestinal obstruction, accompanied by fever, anemia, etc., with a slow course and a tendency to recur.

　　What are the symptoms of idiopathic retroperitoneal fibrosis? Briefly described as follows:

　　1. The early symptoms of idiopathic retroperitoneal fibrosis (RPF) are insidious, mainly manifested as non-specific back pain, abdominal pain, and costal pain, presenting as persistent dull or hidden pain, which can occur at any age, even in newborns, but it is more common in middle-aged people, with twice as many male patients as female patients, and both white and black races can be affected. The disease usually has an insidious onset, a long course, and diagnosis is often made after several months or even years after the appearance of some vague symptoms, with pain being the most common and also the earliest symptom, usually felt as a dull pain or discomfort in the lower lateral abdomen, lumbar sacral region, or lower abdomen; other symptoms include anorexia, weight loss, and fatigue, with possible unilateral or bilateral leg swelling, scrotal swelling or moderate fever, and occasionally palpable masses in the abdomen or pelvis.

　　2. In the advanced stage, the clinical manifestations are often symptoms of compression or involvement of adjacent organs, such as the narrowing of the ureter can cause proximal infection or dilation, which can produce lumbar or costovertebral angle pain, frequent urination, and increased nocturia; compression of both ureters can lead to sudden anuria; due to frequent hydronephrosis or kidney infection, lumbar tenderness is very common; hypertension is common (one of the causes of headache), mostly due to renal obstruction, because with the rupture of the ureter, after the release operation or resection of the non-functional kidney, blood pressure can return to normal, gastrointestinal symptoms may be related to uremia or direct damage to the gastrointestinal tract (such as displaced stenosis).

　　3. There have been reports of biliary and pancreatic duct stenosis, which, if involving the portal vein or splenic vein, can lead to portal hypertension, esophageal varices, and ascites; due to fibrosis, there may be obstruction of retroperitoneal or mesenteric lymphatic drainage, leading to protein-losing enteropathy or malabsorption; compression or obstruction of retroperitoneal lymph nodes, veins, or small arteries can cause unilateral or bilateral leg swelling, penile swelling or scrotal edema, even with abdominal wall varices or varicosities, thrombosis of the lower extremities, weak pulses in the lower extremity distal ends, intermittent claudication, and may be accompanied by fibrosis in other parts (such as mediastinal bile ducts), even硬化性胆管炎, Peyronie's disease (Peyronie's disease, penile cavernosal sclerosis, producing fibrotic painful penile erection, i.e., fibrotic cavernitis).

　　4. During physical examination, there is often tenderness in the lower abdomen and腰部, there may be percussion pain or palpable enlarged kidneys in the renal area, retroperitoneal fibrosis scars are generally not palpable, and hypertension may be accompanied.

　　How to prevent idiopathic retroperitoneal fibrosis? Briefly described as follows:

　　In addition to immunological factors, certain drugs are also pathogenic factors for this disease, such as ergot derivatives, various anesthetics, analgesics, etc. Therefore, effective prevention should be carried out according to the etiology. Retroperitoneal fibrosis is a disease with certain self-limitation and slow progression, but the prognosis is good. The lesions caused by ergot derivatives will reverse after discontinuation of medication. If diagnosed in time, obstruction is relieved, and long-term remission can be achieved. The mortality of retroperitoneal fibrosis is about 9%, and the cause of death is usually renal insufficiency, often due to delayed diagnosis.

　　What examinations should be done for idiopathic retroperitoneal fibrosis? Briefly described as follows:

　　1. Laboratory examination

　　Laboratory examinations for this disease often show accelerated erythrocyte sedimentation rate, varying degrees of anemia, and increased white blood cell count, occasionally with increased eosinophils, and increased alpha and lambda globulins in protein electrophoresis. Urinalysis can be normal or have a small number of white blood cells and red blood cells, and later there may be uremia. Therefore, when the urine of a uremic patient is normal, attention should be paid to whether it is caused by retroperitoneal fibrosis.

　　2. X-ray examination

　　X-ray urography can show displacement of one or both ureters, and the diagnostic significance is the gradual narrowing of the middle segment of the ureter with segmental stricture, which is different from the stricture caused by tumors or calculi, the latter has irregular stricture without gradual narrowing. When the digestive tract is involved, double-contrast X-ray enterography can show segmental stricture in the involved intestines such as the duodenum. Pelvic fibrosis can lead to rectal stricture and straightening, accompanied by an elevated bladder in the shape of a teardrop. Venography can show stenosis of the inferior vena cava or iliac vein.

　　3. CT and MRI

　　Most can find fibrous plaques or abnormal soft tissue masses, and the enhanced scan shows a more intense fibrous tissue sign. Due to the lateral development of fibrosis, the angle between the aorta and the left lumbar great muscle, and the inferior vena cava and the right lumbar great muscle changes, and the dilatation of the proximal ureter can also be displayed. Since MRI is not superior to CT in displaying fibrous plaques, but it can display the changes in blood flow velocity, CT examination is preferred, and MRI is selected for further understanding of hemodynamic changes.

　　4. B-ultrasound examination

　　The mass of this disease is hypoechoic or anechoic, without characteristic manifestations, and the degree of urinary tract obstruction and renal pelvis hydrops can still be observed.

　　First, postoperative dietary formula for retroperitoneal fibrosis:

　　1. Take 4 grams of Jilinshen and 3 grams of American ginseng to stew lean meat.

　　2. Take 4-5 shiitake mushrooms to stew lean meat or chicken breast (drink the soup).

　　3. Take 15 grams of Beiqi, 21 grams of dangshen, 30 grams of Huai Shan, and 15 grams of lotus seeds to stew lean meat.

　　4. Take 30 grams of Tufuling, 30 grams of raw Yiren, and 3 pieces of Yuanro to stew grass carp or water turtle.

　　5. Take 17 grams of dangshen, 21 grams of Zishizi, 10 grams of Jiazi, and 15 grams of Yiren to stew lean meat or chicken.

　　6. Take 3 grams of Tianqi and 3 grams of ginseng (or red ginseng) to stew lean meat or chicken.

　　Two, what foods are good for retroperitoneal fibrosis:

　　On the second day after surgery, some easily digestible foods such as thin rice porridge, soft egg custard, milk, dragon beard noodles, and noodles can be eaten. It is also possible to add one or two meals of full nutrition, such as special medical purpose foods, enteral nutrition agents. This is beneficial for the absorption of nutrients in the body and does not increase the burden on the gastrointestinal tract. However, the digestive ability of the body decreases when just starting to eat, and one should not be in a hurry. Eat less and more often, 5-6 times a day. If the tolerance of the body is enhanced and appetite increases, it is necessary to resume normal dietary intake as soon as possible and eat more foods rich in high-quality protein and vitamins.

　　Three, what foods should not be eaten for retroperitoneal fibrosis:

　　1. Avoid stimulants such as coffee.

　　2. Avoid spicy and刺激性 foods such as scallions, garlic, ginger, and cinnamon.

　　3. Avoid smoking and drinking.

　　4. Avoid greasy, fried, moldy, and salted foods.

　　5. Avoid foods that cause heat, such as rooster, goose, etc.

　　What are the treatment methods for idiopathic retroperitoneal fibrosis? The following is a brief description:

　　1. Corticosteroids

　　Early application of corticosteroids can take effect within a few weeks, and even make the mass significantly shrink or disappear. For patients with mild to moderate urinary tract lesions, the elderly, the weak, or those with systemic diseases, the use of prednisone类药物 is more appropriate. It is also sometimes used for preoperative preparation or postoperative prevention of recurrence. The initial dose is 30-60mg prednisone or prednisolone per day, and the dose is gradually reduced to the minimum effective maintenance dose after the condition is stable, for at least 3 months. Some people have achieved good results by combining hormones and azathioprine, and the efficacy of radiotherapy is not yet certain.

　　2. Surgical operation

　　Although fibrosis itself rarely requires surgical resection, once a large amount of fibrosis occurs, hormone therapy is often ineffective. When the organ is compressed and affects its function, surgery is required. A single bilateral ureterolysis can be performed, and the omentum can be used to wrap the ureter, and the ureter can be moved laterally, which can achieve a good sustained relief. Simple ureterolysis has a high recurrence rate.

　　In advanced stages, for severe urinary tract obstruction, percutaneous nephrostomy drainage can be performed, which is superior to retrograde ureteral catheterization or stenting. It can not only alleviate symptoms in time but also monitor renal function through urine electrolyte testing, allowing most patients to avoid hemodialysis.