A case of multifocal fibrosclerosis involving posterior mediastinal fibrosis, retroperitoneal fibrosis, and a left seminal vesicle with elevated serum IgG4.
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Anti-Inflammatory Agents↗
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Results for “RETROPERITONEAL FIBROSIS”
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Hashimoto's thyroiditis and Graves' disease associated with retroperitoneal fibrosis.
Retroperitoneal fibrosis is a rare disease of uncertain pathogenesis. However, its possible association with several immunopathologic conditions, the possibility of systemic involvement by the fibrous process, the presence of various autoantibodies, and the frequent response to immunosuppressive treatment all support an autoimmune pathogenesis. Riedel's thyroiditis is a rare disease the pathogenesis of which is also thought to be immune-mediated based on its optimal response to steroids; Riedel's thyroiditis is also frequently reported in association with retroperitoneal fibrosis. We describe here two cases of autoimmune thyroid disease associated with retroperitoneal fibrosis, the first with features of primary myxedema, the second of primary thyrotoxicosis. Histology of retroperitoneal fibrosis is documented and it is compatible with an immunopathologic condition. Thus, these two cases add further support to the hypothesis of an autoimmune pathogenesis of retroperitoneal fibrosis and indicate the importance of carefully monitoring for the development of other autoimmune disorders, i.e., of the thyroid gland, in patients with retroperitoneal fibrosis.
Aged↗
Retroperitoneal fibrosis.
Retroperitoneal fibrosis is a rare disease but is important surgically because it can produce ureteral obstruction and renal failure. A wide variety of inflammatory, infective and neoplastic conditions can result in secondary retroperitoneal fibrosis and it is essential to look carefully for an underlying neoplastic condition in all cases of retroperitoneal fibrosis. Idiopathic retroperitoneal fibrosis occurs predominantly in men and results in some form of renal failure in 75% of patients. The classic triad of medial deviation of ureters, ureteral narrowing at L4-5 and proximal hydronephrosis is usually seen in high-dose intravenous pyelograms and the ease of retrograde ureteral catheterization is often diagnostic. Management depends on the degree of renal impairment, with laparotomy, multiple biopsies and ureterolysis being the treatment of choice. Steroids are beneficial, if used early, and the prognosis is excellent if there is no underlying malignant condition.
Humans↗
Retroperitoneal fibrosis.
Retroperitoneal fibrosis encompasses a range of diseases characterised by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighbouring structures--eg, ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Idiopathic disease was thought to result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta, but clinicolaboratory findings--namely, the presence of constitutional symptoms and the high concentrations of acute-phase reactants--and the frequent association of the disease with autoimmune diseases that involve other organs suggest that it might be a manifestation of a systemic autoimmune or inflammatory disease. Steroids are normally used to treat idiopathic retroperitoneal fibrosis, although other options--eg, immunosuppressants, tamoxifen--are available. The outlook is usually good, but, if not appropriately diagnosed or treated, the disease can cause severe complications, such as end-stage renal failure. Here, we review the different aspects of retroperitoneal fibrosis, focusing on idiopathic retroperitoneal fibrosis and on the differential diagnosis associated with the secondary forms.
Adrenal Cortex Hormones↗
Retroperitoneal fibrosis.
Retroperitoneal fibrosis is an uncommon inflammatory disease that leads to extensive fibrosis throughout the retroperitoneum. The majority of cases are idiopathic. The characteristic perivascular distribution of the idiopathic form supports the theory that the disease is an immune-mediated response to severe atherosclerosis. Evidence suggests that idiopathic retroperitoneal fibrosis may be part of a systemic fibrosing disease. Other less common forms include methysergide-related and malignant retroperitoneal fibrosis. The signs and symptoms of the disease are vague and nonspecific, and therefore the diagnosis relies heavily on radiologic findings. Once the diagnosis is suggested, the distinction must be made between malignant and nonmalignant retroperitoneal fibrosis, because the prognosis is dismal for malignant retroperitoneal fibrosis, but very good for other forms of the disease. Surgical biopsy remains the only way to definitively establish this diagnosis. Treatment may be surgical or medical, with the best outcome observed in patients receiving both.
Diagnosis, Differential↗
Spinal cord compression secondary to idiopathic retroperitoneal fibrosis.
Retroperitoneal fibrosis is a rare disorder in which the abnormal fibrotic tissue compresses retroperitoneal organs. In the majority of patients no obvious cause can be found, hence the name idiopathic retroperitoneal fibrosis. The process can also arise from other anatomic areas, suggesting a multifocal origin. We report a case of dorsal epidural compression in a 63-year-old patient harboring idiopathic retroperitoneal fibrosis. A magnetic resonance imaging scan revealed the precise location of the lesion. It is suggested that retroperitoneal fibrosis should be considered in the differential diagnosis of epidural spinal cord compression.
Female↗
Laparoscopic diagnosis of malignant retroperitoneal fibrosis.
Retroperitoneal fibrosis may be caused by a number of disease processes, both benign and malignant. Traditionally, open exploration and biopsy has been performed to exclude a malignant etiology prior to ureterolysis. We report a patient in whom a diagnosis of malignant retroperitoneal fibrosis was confirmed using laparoscopy. In patients in whom a metastatic etiology for retroperitoneal fibrosis is suspected, laparoscopy should be performed and representative biopsies taken. Laparoscopic techniques offer the urologist the ability to obtain an adequate tissue biopsy with a reduction in postoperative morbidity and convalescence associated with traditional laparotomy.
Biopsy↗
[Problem of intestinal compression due to retroperitoneal fibrosis].
Retroperitoneal fibrosis causes almost without exception ureteral stenosis, frequently vascular constriction but rarely intestinal obstruction. Of 17 cases reported in the literature, 14 showed a colon sigma stenosis and 3 an obstruction of the duodenum. The appearance of intestinal stenosis neither correlates with the duration of symptoms nor with the extent of retroperitoneal fibrosis. Of our own cases, 4 out of 48 patients with retroperitoneal fibrosis presented a colon sigma constriction; of 17 patients with retroperitoneal fibro-carcinomatosis 2 had a stenosis of the sigma and one an obstruction of the duodenum. Discussed in reference to the literature and to two typical cases are difficulties in surgical intervention, the emergency situation of intestinal occlusion and the success and limitations of cortisone therapy for moderate intestinal constriction.
Colonic Diseases↗
Familial retroperitoneal fibrosis.
Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.
Child↗
[Treatment outcomes in primary and secondary retroperitoneal fibrosis].
Retroperitoneal fibrosis (RPF) is an uncommon inflammatory disease of the retroperitoneum leading to extensive fibrosis with consecutive obstruction of adjacent organs, namely the ureters. Since no consensus on the standard therapy exists, aim of the current study was to evaluate the outcome of 39 patients with RPF. Between 1986 and 1997 39 cases of RPF were diagnosed: 21 cases had primary RPF and 18 patients had secondary RPF after aortofemoral graft (n = 13), radiation (n = 2), or prior retroperitoneal surgery (n = 2). 21 patients demonstrated unilateral and 16 cases had bilateral hydronephrosis, in 2 patients no dilatation was observed. In 28 cases (n = 12 primary RPF, n = 16 secondary RPF) initial management consisted of DJ-stent placement, whereas in 11 cases (n = 9 primary RPF, n = 2 secondary RPF) percutaneous nephrostomy had to be placed. All patients received oral immunosuppressive agents (prednisolone 1 mg/kg, azathioprine 1 mg/kg/day) for 3 months before reevaluation was performed. In case of complete remission, immunosuppressive medication was continued for another 3 months, in case of stable disease or progression surgery was performed. In 26 cases (n = 15 primary RPF, n = 11 secondary RPF) ureterolysis with intraperitoneal displacement and omental wrapping was performed. 3 patients demonstrated complete remission after oral prednisolone/azathioprine; in 2 cases RPF presented as pelvic mass and was resected followed by immunosuppressive therapy, in another 2 cases bilateral ileal replacement of the ureters had to be performed and 4 cases remained on DJ-stents and nephrostomy, resp. Postoperatively, all patients with primary RPF were continued on immunsuppressive medication for another 3 months. After a follow-up of 6 to 120 months only 3 patients developed a retroperitoneal recurrence and were treated by unilateral nephrectomy or DJ stent placement (n = 2). Our data suggest that the combination of both immunosuppressive medication and surgical management results in an excellent longterm outcome in idiopathic retroperitoneal fibrosis with a recurrence rate of only 8%. Combination therapy should be considered as therapeutic option early in the course of the disease. Primary reconstructive surgery appears to be the most promising approach in secondary retroperitoneal fibrosis with a recurrence rate of only 5%; short external compression of the ureter might be managed by endoluminal balloon dilatation.
Adult↗
Retroperitoneal fibrosis.
Retroperitoneal fibrosis is the formation of a fibrotic plaque in the prelumbar or presacral area which becomes clinically significant when it produces ureteral obstruction. It can occur as a result of taking certain drugs, retroperitoneal fluid collections, inflammatory disease of various intraabdominal organs, previous surgery or radiation therapy, and various metastatic neoplasms. The majority of the causes of retroperitoneal fibrosis are considered idiopathic.
Humans↗
[Idiopathic retroperitoneal fibrosis].
Retroperitoneal fibrosis is an uncommon disease, characterized by the replacement of normal retroperitoneal tissue with fibrosis and/or chronic inflammation. In two thirds of the cases retroperitoneal fibrosis is idiopathic (IRF), whereas in the remaining ones it is secondary/associated to cancer, infections, drugs, autoimmune disease and vasculitis. IRF appears as a dense, fibrous plaque that usually arises between the level of the lower aorta and the common iliac arteries. As the plaque progresses, it engulfs the adjacent structures (e. g., ureters). In its early stages IRF is characterized by a rich infiltrate of lymphocytes, plasma cells and macrophages interspersed within fibroblasts and collagen bundles. In its advanced stages it becomes relatively avascular and acellular with abundant collagen bundles and scattered calcifications. The pathogenesis is unknown: some Authors suggest that IRF is a consequence of a local autoimmune reaction against atherosclerotic plaque antigens whereas others propose that it is the manifestation of a systemic autoimmune disease. The presenting signs and symptoms are non-specific; systemic manifestations (fever, anorexia, weight loss), often associated with local symptoms, are usually found to be related to the entrapment of retroperitoneal structures. The most common local symptom is lumbar and/or abdominal pain. The treatment can be surgical and/or medical: the former is required when obstructive complications are present; the latter, associated or not with surgery, can significantly improve the outcome of IRF patients and usually modifies the natural history of the disease. Steroids and tamoxifen are the most used drugs, whereas other agents such as azathioprine, methotrexate and cyclosporine are usually given to non-responder patients.
Humans↗
Tamoxifen for retroperitoneal fibrosis.
Retroperitoneal fibrosis is characterised by diffuse inflammatory proliferation of fibroblasts and the deposition of collagen fibrils throughout the retroperitoneum, especially in perivascular sites. This results in blockage of the ureters and other tubular retroperitoneal structures such as the vasculature and lymphatic channels. Left untreated, it typically follows a progressive and fatal course. The condition responds favourably to tamoxifen and several case reports describe an improvement in renal function on this therapy. This case report describes a subjective and objective functional improvement of lymphoedema secondary to retroperitoneal fibrosis with the use of tamoxifen.
Antineoplastic Agents, Hormonal↗
Childhood retroperitoneal fibrosis.
Retroperitoneal fibrosis is rarely considered in the differential diagnosis of ureteral obstruction in children even when clinical presentation and radiologic findings are typical. In a 12-year-old boy admitted with a 2-week history of flank pain computed tomography showed an enhancing mass obstructing the left ureter. Pathologic examination of the mass and adjacent segment of ureter revealed retroperitoneal fibrosis.
Child↗
Tamoxifen for retroperitoneal fibrosis.
Retroperitoneal fibrosis, either idiopathic or associated with aortic inflammatory aneurysm, is a chronic fibrotic disease that causes progressive obstruction of ureters and vessels around the lower abdominal aorta. Treatment is often difficult (surgery) or hazardous (steroids). We report a case of a woman with retroperitoneal fibrosis associated with aortic inflammatory aneurysm, who was successfully treated with oral tamoxifen.
Aortic Aneurysm, Abdominal↗
Retroperitoneal fibrosis.
Retroperitoneal fibrosis, an uncommon and ill-defined condition, has a variety of causes and presenting features. The fibrotic process often produces ureteral obstruction and compression of surrounding structures. Pain in the flank, lower abdomen or lumbosacral region is the most common presenting symptom. Certain drugs, neoplasms, retroperitoneal injury or infections may all initiate the fibrotic process. This article describes a case of retroperitoneal fibrosis in a 34-year-old man who had been receiving a beta-adrenergic blocking agent.
Adult↗
The urographic appearances in acute and chronic retroperitoneal fibrosis.
Retroperitoneal fibrosis is a disease which causes renal failure by obstruction of the ureters. The fibrosis may be benign or secondary to malignant disease. The clinical and radiological features of 30 cases have been reviewed. The symptoms are non-specific and diagnosis is often difficult. The most important features are back pain associated with a high ESR. Urography is diagnostic; the well known features are obvious dilatation of the pelvicalyceal system and ureter above the level of the obstruction. However, there is a spectrum of appearances corresponding to the duration of the disease and there may be only minimal dilatation of the pelvicalyceal system or no pelvicalyceal opacification at all. The progress of the disease is variable. Slow progress leads to chronic obstruction and chronic ill health. With more rapid progression, the patient may present with acute obstruction in anuria and, in such cases, urgent ureterolysis is necessary to conserve nephrons. Benign and malignant retroperitoneal fibrosis are often indistinguishable, clinically and radiologically, and laparotomy with biopsy is essential for diagnosis.
Acute Disease↗
Gallium-67 imaging and computed tomography in early retroperitoneal fibrosis.
Retroperitoneal fibrosis is characterized by symptoms associated with gradual compression of retroperitoneal structures. This case report demonstrates the use of Ga-67 imaging and CT in the early diagnostic work-up of this disorder.
Female↗