18:23,20,May,2009 | (922/0/0) | Original

retroperitoneal fibrosis emedicine


Retroperitoneal fibrosis
Luo Chenghua
Retroperitoneal fibrosis (Retroperitoneal Fibrosis, RF), by French urologists Albarran was first reported in 1905, describes the extensive retroperitoneal fibrosis in 1 case combined surgical treatment of patients with ureteral obstruction. Later, in French and German occasionally reported in the literature until 1948, Ormond disease in 2 cases reported in English only after it has been generally recognized national doctors. Beijing Millennium Monument Luocheng Hua Hospital, Colorectal & Anal Surgery
Pathology
Gross pathology
In general, consistent with retroperitoneal fibrosis and dense white fibrous plaque system, usually derived from the aortic bifurcation or the bottom, near the sacral promontory, surrounded aorta can continue to develop and inferior vena cava. Not only distributed along the retroperitoneal spread around the vessels and also in the basin surrounding the iliac bifurcation along the ventral aorta blood vessels, gonadal vessels can still be involved. A few cases, lesions along the celiac artery and superior mesenteric artery forward spread. Fibrosis often spread to both sides, sometimes violations of the ureter and the psoas muscle. Parcels and fibrous ureteral hydronephrosis caused by retroperitoneal fibrosis is a sign. Disease often involving the ureter in 1 / 3, but both sides of any section of the ureter may be involved.
Renal fibrosis is usually located between the door to the basin margin retroperitoneal, and mediastinal fibrosis may transdiaphragmatic extension phase. At least 1 case report, distributed in retroperitoneal fibrosis from the aortic root to the bifurcation of the wide range. Retroperitoneal fibrosis can still be spread to the small bowel mesentery, duodenum, colon, bladder and rare epidural space.
Benign and malignant retroperitoneal fibrosis in general can not be identified.
Histology
Ormond, in its initial report of retroperitoneal fibrosis was observed histological features are: new inflammation and scarring caused by inflammation of the past. He proposed the essence of retroperitoneal fibrosis is the inflammation, the result was later ergobasine itchinson two angles further confirmed.
Early retroperitoneal fibrosis, a chronic active inflammation, a large number of lymphocytes, plasma cells, macrophages, scattered fibroblasts and collagen bundles between the cytoskeleton, there are a few eosinophils, usually no neutral cells, the organization is still There are many small vessels with diffuse distribution. Fibroblasts can develop advanced scar tissue becomes relatively avascular no cells scattered between collagen bundles in the state of calcification.
In patients with biopsy method has been confirmed using continuous active inflammation from fibrosis progression to the other, has been observed in active inflammation and fibrosis in a patient simultaneously in the.
Malignant retroperitoneal fibrosis only difference appears t
o inflammatory infiltration of malignant cells scattered in the nest, the full differentiation of malignant cells often easy to make a diagnosis of primary malignant tumors.
In many cases, the disease follows severe aortic sclerosis, often middle atherosclerotic plaques by loose fibrous outer convex, the aortic wall with chronic inflammatory cell infiltration.
Retroperitoneal fibrosis and sometimes invading into adjacent skeletal muscle, retroperitoneal large veins can be involved, fibrous intimal thickening, and sometimes can cause complete obstruction of veins. Aortic lymph node near the (tube) and sometimes in the mass of occlusion. Ureter often edema, lymphocyte infiltration in the mucosa, but not because of fibrosis and occlusion.
Etiology
A number of factors can induce retroperitoneal fibrosis, including: bleeding, urine leakage, radiation, surgery, non-specific inflammation of the gastrointestinal tract such as Crohn's disease, appendicitis and diverticulitis, and many infections, including tuberculosis, histoplasmosis disease, syphilis and actinomycosis, the fibrosis caused by the location and cause of the performance of a wide variety, and retroperitoneal fibrosis characteristic obvious difference between the para-aortic fibrosis.
2 / 3 of patients with true idiopathic retroperitoneal fibrosis, known as idiopathic retroperitoneal fibrosis, and the rest were with the application of dimethyl ergometrine, cancer and abdominal aortic aneurysm related.
Retroperitoneal fibrosis may be part of systemic fibrotic diseases, there are four kinds of disease associated with retroperitoneal fibrosis, and its performance is quite consistent with histology, which is considered to be the same disease in different parts of the performance: â‘  orbital fibrous pseudotumor , â‘¡ Reid's ear (Riedel's) thyroiditis, â‘¢ sclerosing cholangitis, â‘£ mediastinal fibrosis. Retroperitoneal fibrosis in all patients, 8% -15% in the peritoneum otherwise fibrosis.
Idiopathic fibrosis
In idiopathic fibrosis, early arterial sclerosis lesions located adjacent to fibrosis initially the most important of the aortic area. Atherosclerotic plaques protruding into the outer membrane, middle aortic damage evident. Aortic wall inflammation and no serious inflammation in atherosclerotic arteries consistent and more serious. This chronic inflammation and even fibrosis in the absence of aortic para-aortic region, suggesting that further development of inflammation caused by aortic adventitial fibrosis. Inflammatory infiltration characteristics of the disease, including infiltration of lymphocytes and plasma cells, suggesting that it is immune mediated.
Retroperitoneal fibrosis and connective tissue of many immune-mediated diseases such as ankylosing spondylitis, systemic lupus erythematosus, Wegener's granulocyte cell histiocytosis, Raynaud's disease, nodular nodosa and systemic vasculitis. There are many findings support a retroperitoneal fibrosis itself the cause of immunization. First of all, free from active inflammation to fibrosis and infiltration of cells in the progress consistent with other autoimmune diseases; Second, laboratory tests such as elevated erythrocyte sedimentation rate, g-globulin test positive for elevated and autoantibodies, and immune causes that is consistent; Third, steroids and other immunosuppressive drugs can alleviate the disease, suggesting that their immune etiology; the fourth, as did many other autoimmune diseases, the disease and the major histocompatibility complex HLA-B27 related.
There is growing evidence reveals: ceroid, a plaque was found in the oxidation of fats and protein polymer, it is possible antigen, ceroid in atherosclerotic plaques are isolated until the middle destruction of party identification by lymphocytes and antibodies. Inflammatory cells around the aorta is mainly B lymphocytes, T helper cells followed. Parums, etc. In patients with severe chronic aortic inflammation was detected in the surrounding ceroid antibodies for IgG. Only when the aorta with isolated middle and outer membrane damage when the antibody was inflammatory and ceroid related. No membrane inflammation, not detected ceroid associated IgG.
Inflammatory infiltration of other cells to regulate the proliferation of stromal fibroblasts source of macrophages in fibroblast growth factor, T cells to produce fibroblast growth factor and collagen synthesis stimulating factor.
Aneurysm next to the fibrosis
The authors note that there is leakage of fluid abdominal aortic aneurysm can result in the corresponding adjacent fibrosis, about 5% -23% of abdominal aortic aneurysm and the aneurysm adjacent to fibrosis. Retroperitoneal fibrosis, a lack of iron in the heme with this match. Para-aortic retroperitoneal fibrosis, inflammatory infiltration are consistent with and may be the same disease process. Retroperitoneal fibrosis, fibrosis and the only difference between adjacent aneurysm is aortic dilatation. Up to 10% of abdominal aortic aneurysm was determined to be inflammatory, and is considered different atherosclerotic aneurysms, however, on histological inflammatory arterial aneurysm and no difference in peritumoral fibrosis. Mitchinson therefore proposed to chronic arterial inflammation around to collectively retroperitoneal fibrosis, fibrosis and aortic aneurysm adjacent inflammatory aneurysms.
Two Corner methysergide-related fibrosis
In 1964, Graham describes a case of migraine, methysergide serving two angles retroperitoneal fibrosis occurs in patients with idiopathic retroperitoneal fibrosis with the same clinical presentation, gross pathology and histological features. Since then, have been useful to other ergot derivatives such as 2 - bromo - lysergic hidden shelters and lysergic acid diethyl amide after reports of retroperitoneal fibrosis.
Second, for some angle ergobasine semi-synthetic derivative of ergot, ergot alkaloids by 5 - hydroxytryptamine receptor sites and become competitive inhibition of 5 - hydroxytryptamine antagonists, can lead to endogenous 5 - HT levels, the elevated level of retroperitoneal fibrosis, endocardial fibrosis and pulmonary fibrosis in carcinoid syndrome related to, but has not found a causal relationship. Two Corner methysergide also led to the rise to 5 - HT release and local inflammatory response secondary to mast cell degranulation. Has been found associated Two Corner methysergide affected not only retroperitoneal fibrosis, also affect the heart, lungs, pleura, large blood vessels and gastrointestinal tract.
Two Corner ergobasine related mechanism of retroperitoneal fibrosis, which may be drug as autoimmune response caused by allergies and allergens results. Another theory says that two angles of ergometrine as a weak vasoconstrictor, can lead to aortic wall damage, or through long-term contraction of vascular or ischemic, with idiopathic retroperitoneal fibrosis, the same mechanism, resulting in around the aorta inflammation and fibrosis.
Taking the second angle of about 1% of the patients ergobasine retroperitoneal fibrosis, although the mechanism is unknown, but the relationship between clear, when after stopping the drug can be automatically relieve the symptoms without further treatment.
There are sporadic reports of other drugs and retroperitoneal fibrosis, including: b-blockers and other anti-allergy (a-methyldopa, hydralazine and reserpine), analgesics (aspirin Lin, phenacetin and codeine) and other non-related drugs (haloperidol and amphetamines). But has not found a causal relationship.
Malignant fibrosis
A small number of patients, fibrosis is the connective tissue of the retroperitoneal metastatic tumor cells in response to the result of hyperplasia. This vicious retroperitoneal fibrosis, and retroperitoneal lymph nodes lateral to the ureter to the different shift, make it to the inside of the shift. The most common cause of such fibrosis and other tumors, including Hodgkin lymphoma, certain sarcoma, carcinoid tumors, and breast, lung, colon, kidney, bladder, prostate and cervical cancer and other organs.
Clinical manifestations
Rare retroperitoneal fibrosis clinic, the incidence rate of 1:200,000. Overall male: female of about 2:1, with the races due to rounding. However, the second angle ergobasine retroperitoneal fibrosis associated dominant female patients, female: male is 2:1. The incidence of malignant retroperitoneal fibrosis there was no gender difference.
Retroperitoneal fibrosis, mostly in the incidence between the ages of 50-70, "20 years and> 70 years old caught a rare disease. Several cases of childhood onset in the literature, 1 stillborn fetus found in the disease reports.
Insidious onset of retroperitoneal fibrosis, the symptoms are not specific. The most common symptoms are vague, non-acute, the site is not limited to back pain, abdominal pain, threat (92%) and abdominal pain may spread distribution of zonal involving abdomen and groin, often accompanied by symptoms of gastrointestinal irritation. Pediatric pain can affect ipsilateral hip or buttocks, hip disorders caused by stretching. Uncertain because of pain may be inflammation, ureteral obstruction or motility disorders. Other common symptoms include weight loss, anorexia, nausea or vomiting, general discomfort and performance of cachexia, which may be related to chronic active inflammation.
Treatment when the clinical signs include high blood pressure and fever. May have the inferior vena cava obstruction, duodenal obstruction, or direct signs of intestinal obstruction. Sometimes there may be an abdominal mass and lower extremity swelling, late may have oliguria and anuria.
Non-specific laboratory abnormalities, including azotemia, anemia (and renal dysfunction is proportional to), erythrocyte sedimentation rate increased. Sometimes elevated neutrophil or pyuria. In recent years, was recommended for alkaline phosphatase disease index, which correlated well with elevated ESR, it was reported in 2 patients, responded to treatment, pending further study.
Because the symptoms are vague and not specific, the diagnosis of retroperitoneal fibrosis is often delay, progressive loss of renal function due to serious illness. Retroperitoneal fibrosis is more easily overlooked in young patients, both its symptoms. The final diagnosis requires the use of imaging findings.
Late complications of retroperitoneal fibrosis by the development of fibrosis caused by difficult to control. Parcel of retroperitoneal fibrosis can cause renal dysfunction ureter and eventually lead to renal failure, inferior vena cava and retroperitoneal lymphatic pressure or blood clots can lead to lower limb edema, scrotal edema, or water caused by the gonadal vessel involvement. Duodenum, biliary tract and pancreas may be affected very few cases. Duodenum, small bowel obstruction can lead to direct involvement, bile duct involvement, gross and histological same performance and sclerosing cholangitis, and jaundice, common, fat layer around the destruction of pancreatic retroperitoneal fibrosis can be mistaken for pancreatic cancer. Involving the large intestine can be expressed as diarrhea, constipation or even intestinal obstruction. Retroperitoneal fibrosis can be expressed as epidural mass, through the neural foramen to the cone spread, causing a corresponding level of spinal cord displacement, distortion and atrophy, resulting in delayed paraplegia.
Imaging
Excretory urography
Retroperitoneal fibrosis due to changes in the urinary tract is relatively common, before the advent of the CT, the diagnosis often based on excretory urography.
Perspective on the edge of ambiguity can be displayed psoas muscle, although this performance is not present in all cases. Retroperitoneal fibrosis contrast the typical triad of urinary tract include: delayed excretion of contrast agent with unilateral (20%) or bilateral (68%), hydronephrosis, ureter, 1 / 3 of the inward shift, L4-5 level changes in external pressure of ureteral tapering stenosis.
Shift to the inside of the ureter was once considered to be characteristic of retroperitoneal fibrosis to change, but this is not found in all patients. Further observation also reveal that nearly 20% of the normal ureter also face the same offset to the inside, it was compared 21 patients with retroperitoneal fibrosis and 60 patients without ureteral retroperitoneal fibrosis patients location and found no significant difference.
Interestingly, in patients with retroperitoneal fibrosis, despite the severe clinical renal failure, but very light ureteral obstruction, suggesting that ureteral obstruction in retroperitoneal fibrosis with ureteral involvement on the normal peristalsis, rather than mechanical obstruction. Histological examination also showed ureteral retroperitoneal fibrosis manifested as edema and inflammation, fibrosis and rare, the findings support this view.
Retrograde pyelography
Retrograde pyelography for the severely impaired renal function, but taboo for intravascular imaging of patients when the diagnosis of retroperitoneal fibrosis, or to further clarify the severity of cases have been confirmed.
Typically, retroperitoneal fibrosis retrograde pyelography excretion urography with the performance of the same, the former rigid state was still capable of that ureter. Interestingly, despite the extensive retroperitoneal fibrosis, but can often thin catheter retrograde through the ureter, renal pelvis and ureter and ureteral retrograde expansion of the conflict is no obstruction of the disease was diagnosed.
Lymphangiography
Lymphangiography in the diagnosis of retroperitoneal fibrosis, excretory urography for the additional measures. The fine structure of retroperitoneal lymphatic vessels than ureteral retroperitoneal fibrosis are more susceptible to pressure, the lymphatic obstruction should occur mainly in the ureter and retroperitoneal vascular obstruction pressure before the change in the urinary tract was made before the film can be in the lymphatic vessels retroperitoneal fibrosis was found. Also reported revealed that very few extensive retroperitoneal fibrosis and severe compression of the ureter patients lymphangiography can be no exception.
Retroperitoneal fibrosis lymphangiography performance, including: contrast agent through the iliac artery or aortic lymphatic next extended, L3-4 level of the fourth lumbar lymphatic flow obstruction caused by lymphatic cone is not above the level of development, the lateral lymphatic filling, regurgitation caused by ectopic lymphatic imaging. Aortic lymph nodes in the mesentery and small appear abnormal filling defect.
Lymphatic making videos without lymph node metastasis can be excluded on the diagnosis of malignant tumors.
CT scan
The current CT scanning has replaced other imaging studies, a diagnosis of retroperitoneal fibrosis patients and follow-up of the preferred method. Not only outside the tumor can show the degree of development of urinary tract, and urinary tract involvement can be displayed in front of retroperitoneal lesions.
CT scan of retroperitoneal fibrosis with different thickness showed para-aortic soft tissue mass, sacral promontory in the kidney between the door and around the aorta and inferior vena cava, can be spread laterally surrounding the ureter led to different degrees of hydronephrosis. Lumps generally do not cause forward displacement of aorta. Between the adjacent psoas muscle mass and fat layer disappears. Few cases, the tumor can be very asymmetric, abnormal position, making diagnosis difficult. CT, 30% of patients still can not find mass, when these patients in the laparotomy limited common pelvic fibrosis and inflammation.
CT of retroperitoneal fibrosis on plain films and muscle tissue density equal to, with varying degrees of progression and showed enhanced. Vascular activity in the early period, CT slices was significantly enhanced, while the latter no small blood vessels of the increase. Enhanced CT scan of the difference between morning and evening as a judge in the course of treatment progress or remission of follow-up means.
Retroperitoneal fibrosis with the CT-chip should first identify the following diseases: retroperitoneal hematoma, primary retroperitoneal sarcoma and retroperitoneal metastases. In addition, at least one case report of retroperitoneal amyloidosis consistent with retroperitoneal fibrosis, in need of identification.
In the differential diagnosis of peritoneal morphological changes after structures have a certain sense, the majority of retroperitoneal tumors outside of the ureter to the shift, and its inward displacement of retroperitoneal fibrosis. Although a few cases of aortic retroperitoneal fibrosis can shift forward, but with obvious aortic often prompted the shift to malignant tumors. Retroperitoneal fibrosis retroperitoneal tumor were different from the other for its failure to produce local bone destruction. CT is used to distinguish between value and enhanced with malignant retroperitoneal fibrosis of little value. Some metastases, particularly those with connective tissue proliferation concerns, such as malignant retroperitoneal fibrosis, CT scan consistent with the performance of fibrous tissue, not with idiopathic retroperitoneal fibrosis identification.
Ultrasound
Ultrasound has been used as diagnostic and follow-up of retroperitoneal fibrosis of non-invasive method, and widely used. Ultrasound examination revealed retroperitoneal fibrosis is a large, clear-cut boundary retroperitoneal smooth mass, center of the sacral promontory, the ring surrounding the aorta and inferior vena cava, involvement spread to both sides of the ureter, and adjacent tissue The inter-face disappeared. Homogeneous mass, often showing hypoechoic. May have different degrees of hydronephrosis and hydroureter.
Doppler ultrasound was an attempt to identify malignant and non-malignant retroperitoneal fibrosis, but it seems that lack of information identifying characteristics. Color Doppler ultrasound in determining the value of retroperitoneal tubular structure.
MR imaging
Ability to multi-axis MRI imaging without contrast agents and iodine can be better used to show blood vessels and other advantages, making it a promising retroperitoneal fibrosis screening method.
The signal intensity of retroperitoneal fibrosis with T1, T2 relaxation time in different adjacent psoas muscle and adipose tissue, so the MRI can better show its shape and scope. Retroperitoneal fibrosis was in the low-density signal on T1-weighted, T2-weighted according to the different course of the disease was different signal strength. Early stage of active inflammation, due to retroperitoneal fibrosis epithelial capillaries within the local connection between the holes, the liquid content within the organization, on the T2-weighted high signal intensity. Fibrosis in the later period, no blood vessels, cell, tissue fluid containing low, T2-weighted and with characteristic on the low signal strength. Similarly, the early stage of steroid therapy to reduce tissue edema, may have demonstrated in the T2-weighted low signal intensity, which can still be used for MR imaging evaluation of treatment response.
Many tumors, especially lymphoma, high on T2-weighted signal intensity, inflammation of retroperitoneal fibrosis is difficult and early tumors. Some authors tried to identify malignant and non-MRI of malignant retroperitoneal fibrosis, although the agreement between the two forms, but the uneven signal intensity on T2-weighted were suggestive of malignant retroperitoneal fibrosis, even so, its sensitivity is still not enough to diagnosis, tissue Science biopsy diagnosis of malignant lesions is the only sure way.
Nuclear Medicine
Retroperitoneal fibrosis in the diagnosis and follow-up, the value of nuclear medicine uncertain. Absorption of retroperitoneal fibrosis has been reported radioactive gallium (Ga). The mechanism of gallium concentration is not clear, one explanation for the inflammatory zone lymphocytes with Ga67, Ga and thus absorb the early inflammatory response to disease. Described one case reported after steroid hormone treatment decreased their intake Ga67, suggesting that nuclear medicine can be used to monitor response to treatment of retroperitoneal fibrosis.
Nuclear medicine may be another valuable application is to identify malignant and non-malignant retroperitoneal fibrosis. In Study 2 - deoxy -2 - (F-18) fluoro-D-glucose (F-18DG) positron emission tomography (PET) in, Kubota and other retroperitoneal fibrosis was observed showing low uptake of F-18DG, and showed high uptake in malignant lymphoma, postoperative scar tissue fibrosis also showed low uptake.
Treatment
Although rare in retroperitoneal fibrosis, if not addressed, the slow progress of inflammation, can plug any hollow organs, particularly the ureter and major blood vessels, and bilateral ureteral involvement can lead to kidney failure and death, so the disease should be subject to clinical attention. Retroperitoneal fibrosis patients for therapeutic purposes include: to establish the possible diagnosis and rule out malignancy, relieve ureteral obstruction and restore renal function, preventing inflammation can cause further obstruction to development.
Methysergide taking two angles should be immediately discontinued, once the withdrawal symptoms often ease, fibrosis regression. Most patients, especially ureteral obstruction in patients with mild renal dysfunction, the condition may be a few days to several weeks to improve, other improvements need no further treatment, such as re-taking the fibrosis can be further recurrence.
Surgical treatment
In the original report in Albarran1905, describes two cases of retroperitoneal fibrosis secondary to ureteral obstruction, successful lysis of the ureter (the ureter dissected out from the surrounding fibrosis). Treatment of retroperitoneal fibrosis is still the most basic surgical treatment.
Traditional surgical treatment of retroperitoneal fibrosis include: laparotomy, more deep retroperitoneal biopsy, and ureteral ureteral lysis shift. Ureteral obstruction as the main performance, advocate the use of ureteral lysis combined with intra-abdominal metastasis, surgical method with good access to long-term renal decompression. Ureteral lysis usually easier, such as lysis difficulties, you should consider the possible malignant lesions. Severe ureteral obstruction in the emergency treatment, can be used such as percutaneous nephrostomy decompression kidney, when increased metabolic disorders, the need to further lysis ureter. Malignant retroperitoneal fibrosis in malignant cells less easily confused with inflammatory infiltration and missed things, so the biopsy should be more deep. Once the ureter free, it should shift to the outside will be placed in the ureter and the retroperitoneal fat between the fibers can also be wrapped omentum fat, ureter, or move it to the abdominal cavity, then stitching close to the retroperitoneum.
Many new methods have been applied to surgery. Surgery can be a Gor-Tex peritoneal membrane placed between the ureter and retroperitoneal not closed peritoneum. When there is infiltration of the ureter, a ureteral resectable and end anastomosis. Lysis when the ureter surgery and hormone failure or difficulties, or have repeatedly damaged the continuity of the ureter obstruction should be considered autologous to the pelvic kidney transplantation. There lysis by laparoscopic ureteral surgery and intraperitoneal built reports dealing with unilateral ureteral obstruction.
Medical treatment
Medical treatment of retroperitoneal fibrosis with chronic inflammation of the purpose is to control. Surgical treatment of retroperitoneal fibrosis only for certain areas, namely, obstruction, it can not control the disease for inflammation and fibrosis, the latter can affect the other retroperitoneal structures. Although nearly 90% after release of ureteral obstruction be lifted, but often re-obstruction occurred, accounting alone for treatment of ureteral release 22% of cases. According to statistics, after surgical treatment alone, the recurrence of retroperitoneal fibrosis was 48%, but steroid therapy combined with surgery the recurrence rate was 10%. Large group of patients showed that hormones increase the depth of surgical treatment for renal failure, severe upper urinary tract dilatation, and urinary tract abscess were.
Application of steroid treatment of retroperitoneal fibrosis first in 1958 by Ross and Tinckler reported. Steroid anti-inflammatory effect and inhibit the ability to mature fibrous tissue in the control of chronic inflammation of retroperitoneal fibrosis have been more ideal. Steroid therapy was 100% effective. In idiopathic retroperitoneal fibrosis, urinary obstruction light, no significant metabolic abnormalities, or systemic symptoms were not significant in light can be treated with steroids alone. Seen within 24 hours of starting treatment, symptoms improved recovery of renal function within 3 days of retroperitoneal soft tissue can be significantly reduced, accompanied by hydronephrosis improved. Although steroid therapy in active inflammation of retroperitoneal fibrosis of effective cells in the late fibrotic stage, little to no benefits.
Dose steroid treatment and more based on experience. Steroid hormone treatment on time, most of the advocates for at least 6 months. The authors believe that further long-term administration of small doses is an effective and safe method. Most of the continuing claims increased ESR is the indication for steroids, and ESR is normal withdrawal indications.
Interestingly, the line of patients with unilateral ureteral lysis in retroperitoneal fibrosis can be observed "spontaneous" improvement, then the contralateral ureteral obstruction "spontaneous" improvement. Sometimes only line of biopsy can make a significant improvement of clinical symptoms of patients. This phenomenon may be the perioperative period due to higher endogenous steroid hormones. Asthma and rheumatoid arthritis patients, this phenomenon also exists, according to reports of steroid hormone levels in plasma during surgery can be increased by 2 times, 1-3 days after surgery at a higher level.
Although the application of steroid treatment of retroperitoneal fibrosis has been shown to be more successful, but its application remains controversial. Medical treatment of retroperitoneal fibrosis often lack the most important drawback is confirmed and excluded from histological evidence of malignancy. Many surgeons believe that biopsy is to exclude malignancy most fundamental way, as objects in a rich inflammatory infiltration of tumor cells in a relatively small, it takes more deep biopsy, CT guided fine needle aspiration or core needle biopsy, for organizing specimens less, and thus the value of small or even feasible. By surgical biopsy will remain even if the initial malignant retroperitoneal fibrosis misdiagnosed as idiopathic (non-malignant) retroperitoneal fibrosis can be diagnosed by a few months later, can be found in a small number of reports.
Steroid therapy with retroperitoneal fibrosis has the following uncertainty advantages: First, the acute patients treated with steroids can ease the swelling caused by ureteral obstruction and improve clinical symptoms and to avoid emergency surgery and elective surgery, ureteral stent was also used as temporary measures to support . Secondly, steroids are also used for adjuvant therapy after surgery, the first prevention of fibrosis, thereby preventing further obstruction and other symptoms. Wagenknecht and Hardy found that corticosteroids as adjuvant treatment of ureteral release the best, but there are still some patients treated with steroids failed to prevent secondary obstruction recurrence. Third, in elderly patients, weak, or explicitly ruled out the possibility of malignant lesions in patients, can be used alone steroid hormone treatment.
Recently, immunosuppressive drugs such as azathioprine and cyclophosphamide, are used for the treatment of retroperitoneal fibrosis, the successful application of these drugs strongly support the autoimmune etiology of this disease is the theory. Tamoxifen has also successfully applied to the disease, although its not clear mechanism of action may be related to an inhibitory growth factor transforming growth factor ─ ─ synthesis and secretion increase related.
Prognosis
The patient's prognosis depends on the doctor's degree of renal dysfunction, urinary infection and the extent of the existence and stability of retroperitoneal fibrosis or the progress.
After surgery, retroperitoneal fibrosis, continued its development of other complications and should therefore be further observation following obstruction of the vena cava or duodenum may occur. CT examination may be on a regular basis In addition, follow-up of renal function, hematocrit, especially ESR, for all patients with a prognostic value, ESR increase reflects some degree of inflammation.
With idiopathic retroperitoneal fibrosis is different from malignant retroperitoneal fibrosis and poor prognosis. Although reported long-term survival, but most patients survive only 3-6 months after diagnosis.
References
1. Graham JR: Methysergide for the prevention of headaches. Experience in 500 patients over 3 years. N Engl J Med270 :67-72, 1964
2. Kotta JJ and Dunnick NR: Retroperitoneal fibrosis. Radiologic Clinics of North America34 :1259-1275, 1996
3. Kubota K, Yamada K, Yoshioka S, et al: Differential diagnosis of idiopathic fibrosis from malignant lymphadenopathy with PET and F-18 fluorodeoxyglucose. Clin Nucl Med17 :361-363, 19992
4. Mitchinson MJ: Chronic periaortitis and periarteritis. Histopathology8 :589-600, 1984
5. Mitchinson MJ: The pathology of idiopathic retroperitoneal fibrosis. J Clin Pathol23 :681-689, 1970
6. Ormond JK: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory process. J Urol59 :1072-1079, 1948
7. Parums DV, Brown DL, Mitchinson MJ: Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med114 :383-387, 1990
retroperitoneal fibrosis emedicine

Log Original: http://luochiang.blog.sohu.com/79359831.html retroperitoneal fibrosis
Luo Chenghua
Retroperitoneal fibrosis (Retroperitoneal Fibrosis, RF), by French urologists Albarran was first reported in 1905, describes the extensive retroperitoneal fibrosis in 1 case combined surgical treatment of patients with ureteral obstruction. Later, in French and German occasionally reported in the literature until 1948, Ormond disease in 2 cases reported in English only after it has been generally recognized national doctors.
Pathology
Gross pathology
In general, consistent with retroperitoneal fibrosis and dense white fibrous plaque system, usually derived from the aortic bifurcation or the bottom, near the sacral promontory, surrounded aorta can continue to develop and inferior vena cava. Not only distributed along the retroperitoneal spread around the vessels and also in the basin surrounding the iliac bifurcation along the ventral aorta blood vessels, gonadal vessels can still be involved. A few cases, lesions along the celiac artery and superior mesenteric artery forward spread. Fibrosis often spread to both sides, sometimes violations of the ureter and the psoas muscle. Parcels and fibrous ureteral hydronephrosis caused by retroperitoneal fibrosis is a sign. Disease often involving the ureter in 1 / 3, but both sides of any section of the ureter may be involved.
Renal fibrosis is usually located between the door to the basin margin retroperitoneal, and mediastinal fibrosis may transdiaphragmatic extension phase. At least 1 case report, distributed in retroperitoneal fibrosis from the aortic root to the bifurcation of the wide range. Retroperitoneal fibrosis can still be spread to the small bowel mesentery, duodenum, colon, bladder and rare epidural space.
Benign and malignant retroperitoneal fibrosis in general can not be identified.
Histology
Ormond, in its initial report of retroperitoneal fibrosis was observed histological features are: new inflammation and scarring caused by inflammation of the past. He proposed the essence of retroperitoneal fibrosis is the inflammation, the result was later ergobasine itchinson two angles further confirmed.
Early retroperitoneal fibrosis, a chronic active inflammation, a large number of lymphocytes, plasma cells, macrophages, scattered fibroblasts and collagen bundles between the cytoskeleton, there are a few eosinophils, usually no neutral cells, the organization is still There are many small vessels with diffuse distribution. Fibroblasts can develop advanced scar tissue becomes relatively avascular no cells scattered between collagen bundles in the state of calcification.
In patients with biopsy method has been confirmed using continuous active inflammation from fibrosis progression to the other, has been observed in active inflammation and fibrosis in a patient simultaneously in the.
Malignant retroperitoneal fibrosis only difference appears to inflammatory infiltration of malignant cells scattered in the nest, the full differentiation of malignant cells often easy to make a diagnosis of primary malignant tumors.
In many cases, the disease follows severe aortic sclerosis, often middle atherosclerotic plaques by loose fibrous outer convex, the aortic wall with chronic inflammatory cell infiltration.
Retroperitoneal fibrosis and sometimes invading into adjacent skeletal muscle, retroperitoneal large veins can be involved, fibrous intimal thickening, and sometimes can cause complete obstruction of veins. Aortic lymph node near the (tube) and sometimes in the mass of occlusion. Ureter often edema, lymphocyte infiltration in the mucosa, but not because of fibrosis and occlusion.
Etiology
A number of factors can induce retroperitoneal fibrosis, including: bleeding, urine leakage, radiation, surgery, non-specific inflammation of the gastrointestinal tract such as Crohn's disease, appendicitis and diverticulitis, and many infections, including tuberculosis, histoplasmosis disease, syphilis and actinomycosis, the fibrosis caused by the location and cause of the performance of a wide variety, and retroperitoneal fibrosis characteristic obvious difference between the para-aortic fibrosis.
2 / 3 of patients with true idiopathic retroperitoneal fibrosis, known as idiopathic retroperitoneal fibrosis, and the rest were with the application of dimethyl ergometrine, cancer and abdominal aortic aneurysm related.
Retroperitoneal fibrosis may be part of systemic fibrotic diseases, there are four kinds of disease associated with retroperitoneal fibrosis, and its performance is quite consistent with histology, which is considered to be the same disease in different parts of the performance: â‘  orbital fibrous pseudotumor , â‘¡ Reid's ear (Riedel
1. Bladder varices - rare cause of painless hematuria . Koshy, CG; Govil, S; Shyamkumar, NK; Devasia, A;
Urology 2009,1
Keywords: Hematuria; Humans; Male;
Abstract: A patient presented to the urology outpatient department with complaints of recurrent episodes of painless hematuria. A contrast-enhanced computed tomography sc . 2. Ultrasonography diagnosis of idiopathic retroperitoneal fibrosis that value Chunguang Chen Yantao Gui-Rong Wang Ying
China Medical Imaging 18 01 2010
Keywords: retroperitoneal fibrosis; idiopathic; ultrasonography;
Abstract: Objective: Idiopathic retroperitoneal fibrosis (IRPF) ultrasound image characteristics and diagnostic value. Materials and Methods: Retrospective analysis of 11 cases IRPF ultrasonographic appearance of patients. Results: 11 patients showed an abdominal aorta around the low . 3. Treatment of idiopathic retroperitoneal fibrosis w. Vega J; Goecke H; Tapia H; Labarca E; Santamarina M; Martinez G;
American Journal of Kidney Diseases 2009,4
Keywords: Adult; Aged; Aged;
Abstract: BACKGROUND: Idiopathic retroperitoneal fibrosis (IRPF) is an uncommon, but increasingly recognized, disease. Autoimmune mechanisms probably participate in its p. 4. Retroperitoneal fibrosis induced acute renal failure in 8 cases of end-Jun Hou
Misdiagnosis of China Volume 10 03 2010
Keywords: retroperitoneal fibrosis / complications; renal failure, acute / etiology;
Abstract: Idiopathic retroperitoneal fibrosis induced acute renal failure is a rare disease of renal medicine, because of its typical clinical symptoms are often misdiagnosed. The author retrospectively analyzed 2005/2009 of 8 cases of acute renal retroperitoneal fibrosis functional decline . 5. Atypical Ormond's disease associated with bile duc . Quante M; Appenrodt B; Randerath S; Wolff M; Fischer HP; Sauerbruch T;
Scandinavian Journal of Gastroenterology 2009,1
Keywords: Anastomosis; Roux-en-Y; Bile Duct Diseases;
Abstract: A 55-year-old woman with suspected hilar cholangiocarcinoma presented with jaundice and dilated intrahepatic bile ducts owing to high-grade hepatic duct conflue . 6. Retroperitoneal fibrosis in 1 case and the recent national clinical analysis of 106 cases reported in the literature Mengyu Jun Wujia Bin Wei Lixin
International Journal of Urology 30, 2010 Volume 02
Keywords: retroperitoneal fibrosis; Retroporitoneal Fibrosis;
Abstract: Objective To understand the retroperitoneal fibrosis (retropeitoneal fibrosis, RPF) of the clinical features and signs of early diagnosis of RPF. Methods 1 case analyzed retrospectively RPF and domestic magazines . 7. Retroperitoneal fibrosis associated with psoriasis . Famularo G; Palmisano A; Afeltra A; Buzzulini F; Versari A; Minisola G; Vaglio A;
Scandinavian journal of rheumatology 2009,1
Keywords: Aged; Aorta; Abdominal;
Abstract: Retroperitoneal fibrosis (RPF) is characterized by the formation of a dense retroperitoneal plaque of a fibro-inflammatory tissue that is often associated with . 8. Retroperitoneal fibrosis in 1 case report of acute renal failure induced flower Lijun Fang Jing Jin-fu
Aerospace Medicine 21 01 2010
Keywords: retroperitoneal fibrosis; acute renal failure;
Abstract: The retroperitoneal fibrosis is a rare chronic disease, the disease can occur at any age, onset occult, often misdiagnosed or missed diagnosis. Now our hospital in 2006-05 due to "renal water, acute renal failure" treated patients do report to the . 9.
Retroperitoneal fibrosis: a review of clinical fea . Cronin, CG; Lohan, DG; Blake, MA; Roche, C; McCarthy, P; Murphy, JM;
American Journal of Roentgenology 2008,2
Keywords: Retroperitoneal Fibrosis; Diagnostic Imaging; Review [Publication Type];
Abstract: OBJECTIVE: Retroperitoneal fibrosis is a rare collagen vascular disorder of unclear cause. Both benign and malignant associations have been described, rendering . 10. Idiopathic retroperitoneal fibrosis induced acute renal failure - report of 3 cases Zheng School Li Xiong root
Modern Medicine 2010 16 14
Keywords: retroperitoneal fibrosis; renal failure; diagnosis;
Abstract: Objective idiopathic retroperitoneal fibrosis (IRF) led to the diagnosis and treatment of acute renal failure. Methods IRF 3 patients in our hospital with acute renal failure patients and review of literature related to IRF patients with clinical manifestations, diagnosis . A total of 246 records 1 / 25 93       . 4:
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