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Results for “Retroperitoneal Fibrosis”

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PubMed1998

Asbestos exposure: a potential cause of retroperitoneal fibrosis.

The etiology of retroperitoneal fibrosis is unknown in 70% of the cases. The aim of our study was to examine the possible association between occupational asbestos exposure and retroperitoneal fibrosis; only two cases have been reported in the literature. We gathered all the cases of retroperitoneal fibrosis diagnosed in the Tampere University Hospital between 1987 and 1995. We examined their hospital records to evaluate the possible etiology of the disease. We also sent a structured questionnaire to all living patients (10/13) to obtain information on their asbestos exposure. The chest radiographs of the patients were re-read to evaluate possible changes resulting from asbestos exposure. We found 13 cases of idiopathic retroperitoneal fibrosis. Seven patients (all male) had been exposed to asbestos in the past. The chest radiographs of the four most-exposed patients showed characteristic asbestos-related abnormalities, including bilateral pleural plaques, round atelectasis and small irregular lung opacities. In our study, we found that asbestos exposure and asbestos-induced changes in the lung and pleura were common among male retroperitoneal fibrosis patients. We suggest that occupational exposure to asbestos may be an important etiological factor for retroperitoneal fibrosis.

Adult↗

PubMed1983

Do beta-adrenoceptor blocking drugs cause retroperitoneal fibrosis?

The aetiology of retroperitoneal fibrosis is unknown. From time to time different drugs have been suggested as a cause, including beta adrenoceptor blocking agents. The notes of 100 hospital inpatients in whom retroperitoneal fibrosis had been diagnosed were surveyed to identify the date of onset of symptoms, the drugs prescribed before then, and the blood pressures on admission. Seventy one patients had diastolic pressures on admission of 90 mm Hg or more and 53 of these had pressures of at least 100 mm Hg despite 14 of the patients taking anti-hypertensive drugs at the time. Six patients had received beta-adrenoceptor blocking agents before the onset of symptoms and a further 24 received them subsequently. In particular, three patients continued treatment for at least six years after the diagnosis was made and surgery performed, without suffering a recurrence of retroperitoneal fibrosis. Besides beta-adrenoceptor drugs, diuretics and other hypotensive drugs were taken in amounts reflecting the pattern of drug use among hypertensive patients generally. Rather than being causal agents, the antihypertensive drugs were probably being used to treat hypertension associated with the retroperitoneal fibrosis, and our findings suggest that beta-adrenoceptor blocking agents do not cause retroperitoneal fibrosis.

Adolescent↗

PubMed2004

Epidemiology, clinical features and treatment of idiopathic retroperitoneal fibrosis: our experience.

Retroperitoneal fibrosis (RF) is an uncommon disease with an estimated incidence of 1 case on 200.000 inhabitants. Idiopathic RF (2/3 of cases) must be distinguished from secondary RF, caused by hexogen substances assumptions, radiotherapy and various diseases. The treatment of RF is not yet univocally defined. From 1984 to 2002 we observed 15 patients (1 case with idiopathic retroperitoneal fibrosis) i.e. 27 ureteral units (1 patient was previously nephrectomized and 2 showed a monolateral ureteral involvement). The mean age was 55 and the male:female ratio was 0.66:1. The commonest presenting symptoms were acute renal failure or renal colic. 14 patients needed preoperatory urinary drainage by nephrostomy or ureteral stenting. 6 patients were treated with steroids or tamoxifen without response so that all the 15 patients underwent surgery. 6 patients were lost to follow-up after 8 months while the other 9 underwent clinical and radiological controls for a mean time of 84 months (range 12-168) with evidence of normal canalization or steady dilatation of the excretory system for 8 of them. 1 patient developed a relapsing ureteral stenosis after ureterolysis and intraperitonealisation and needed ureterectomy with ileal interposition. In a case with monolateral presentation a controlateral stenosis was evidenced 5 years later. According to literature data, the surgical technique that ensured us the best results was ureterolysis with omental wrapping of the ureter. In our experience, medical treatments gave poor outcomes. However, in consideration of the low incidence of RF, perspective and multicentric studies are necessary to establish which is the most suitable treatment for each patient. Follow-up must be extended in patients with monolateral presentation to precociously reveal a possible contralateral involvement.

Adult↗

PubMed1985

[Computerized tomography in the diagnosis and follow-up of retroperitoneal fibrosis].

We report 4 patients with surgically proved idiopathic retroperitoneal fibrosis. Retroperitoneal fibrosis was diagnosed correctly in 3 of the patients by computerized tomography. On the computerized tomogram, retroperitoneal fibrosis demonstrated a characteristic soft tissue mass enveloping the vessels and ureters. In 2 patients, the follow-up computerized tomogram revealed spontaneous resolution of the mass in the retroperitoneal space. Computerized tomography is one of the most useful methods in the preoperative evaluation and postoperative follow-up of this disease.

Aged↗

PubMed1991

Retroperitoneal fibrosis accompanying immune thrombocytopenia.

Retroperitoneal fibrosis represents an idiopathic systemic fibrosing syndrome which leads to the development of progressive hydronephrosis and azotemia (Stewart and Friberg: Southern Medical Journal 77:1185-1187, 1984; Morad et al.: American Journal of Medicine 82:363-366, 1987). Evidence in the literature supports a hypothesis that retroperitoneal fibrosis represents a systemic autoimmune process (Lichon et al.: Journal of Rheumatology 11:373, 374, 1984; Lipman et al.: JAMA 196:204-206, 1966; Littlejohn and Keystone: Journal of Rheumatology 8:665-669, 1981; Katz et al.: American Journal of Clinical Pathology 67:436-439, 1977). We report a case of a patient with retroperitoneal fibrosis, immune thrombocytopenia, presence of antinuclear antibody, and direct Coombs positivity which provides further evidence that retroperitoneal fibrosis is an autoimmune process. Progressive obstructive uropathy related to his fibrosis led to bilateral hydronephrosis and significant renal function impairment. Also, he developed marked immune thrombocytopenia. Treatment with prednisone produced a dramatic response in both platelet count and renal function.

Aged↗

PubMed1991

Treatment of idiopathic retroperitoneal fibrosis by immunosuppression.

Idiopathic retroperitoneal fibrosis is exceedingly uncommon in childhood and its etiology is uncertain. Support for an immunological basis for the disease is given by a report of a 14-year-old girl with severe retroperitoneal fibrosis causing progressive azotemia in whom azathioprine and prednisolone were used successfully. This case supports the efficacy of immunotherapy in the treatment of idiopathic retroperitoneal fibrosis.

Adolescent↗

PubMed2006

IgG4-associated multifocal systemic fibrosis complicating sclerosing sialadenitis, hypophysitis, and retroperitoneal fibrosis, but lacking pancreatic involvement.

A 71-year-old man was admitted with malaise, mild fever, anorexia, body weight loss, lower back pain, thirst, and polydipsia. He showed bilateral swelling of the submandibular glands. Examinations showed panhypopituitarism and a high serum IgG4 concentration. Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed uptake in the pituitary gland, bilateral submandibular gland, bilateral hilar and mediastinal lymph nodes, and a mass consistent with retroperitoneal fibrosis, but not in the pancreas. Biopsy specimens from the submandibular gland and retroperitoneal mass indicated sialadenitis and retroperitoneal fibrosis respectively, and showed severe fibrosis and inflammation with marked lymphoplasmacytic infiltration and IgG4-positive plasma cell infiltration. Hormone replacement therapy with hydrocortisone resulted in marked clinical improvement. Systemic involvement found in this patient possibly corresponded to the new concept of IgG4-associated multifocal systemic fibrosis.

Aged↗

PubMed2001

Retroperitoneal fibrosis with duodenal stenosis.

Retroperitoneal fibrosis is a rare disease characterized by the formation of dense plaque of fibrous tissue covering the retroperitoneal structures. This disease is commonly presented as ureteral obstruction, but the involvement of duodenum is rare. We report a case of retroperitoneal fibrosis which was complicated with duodenal stenosis and was successfully treated with corticosteroids. A 58-yr-old man, who had history of aorto-iliac bypass graft due to arteriosclerosis obliterans with infrarenal aortic occlusion was admitted to the hospital with abdominal pain and a mass. Abdominal CT scan revealed the periaortic soft tissue mass encircling grafted aorta and stenosis of duodenal third portion. Retroperitoneal fibrosis with duodenal stenosis was diagnosed and prednisolone therapy was initiated. Follow-up CT scan showed that the patient responded to prednisolone therapy with eased pain, shrinking periaortic mass, and reduced duodenal stenosis.

Anti-Inflammatory Agents↗

PubMed2000

The role of advanced atherosclerosis in idiopathic retroperitoneal fibrosis. Analysis of nine cases.

Idiopathic retroperitoneal fibrosis is an uncommon inflammatory disease characterized by a periaortic fibrous mass throughout the retroperitoneum that often surrounds the ureters, leading to ureteral obstruction. An immune-mediated process has been suggested in which the antigen is derived from atherosclerotic plaques. The optimal treatment of patients with idiopathic retroperitoneal fibrosis is unknown because of its rare occurrence. Surgery, medical treatment with immunosuppressive drugs, or a combination of both are proposed. In this retrospective study we report nine cases of idiopathic retroperitoneal fibrosis treated in our hospital. We quantitated the severity of atherosclerosis by measuring the degree of calcification in the aorta and compared this with a group of 19 age- and sex-matched controls without idiopathic retroperitoneal fibrosis. The idiopathic retroperitoneal fibrosis patients had a higher percentage of aneurysms of the abdominal aorta. However, abdominal aortic calcifications are not more prominent in idiopathic retroperitoneal fibrosis patients than in age-matched controls. These findings suggest that atherosclerosis as determined by abdominal aortic calcifications is not enough to provoke idiopathic retroperitoneal fibrosis. Furthermore, we evaluated the relapse rate of surgery versus medical treatment. In contrast to recent publications, no superiority of corticosteroids over surgical treatment could be demonstrated. However, our study is too small and retrospective. We estimate that a study which could find the most optimal treatment for idiopathic retroperitoneal fibrosis would need the participation of 40-50 medical centers each with a catchment population of about 200,000.

Adult↗

PubMed1977

Peripheral ischemia due to retroperitoneal fibrosis.

Two patients with retroperitoneal fibrosis demonstrated symptoms of peripheral vascular ischemia. Arteriolysis to free the distal aorta and iliac vessels was successful in both patients. Aortography in the posteroanterior view was equivocal, but the accompanying urologic abnormalities and a history of methysergide ingestion helped establish the etiology of the ischemia. The most direct approach to the treatment of the vascular obstruction caused by retroperitoneal fibrosis is complete arteriolysis. Most previous reports indicate that it is relatively easy to establish a dissection plane between the fibrotic plaque and the vessel wall. We found that the fibrotic process invaded the vessel wall. Accordingly, the surgeon must anticipate a difficult, tedious dissection when performing arteriolysis for the treatment of vascular compression secondary to retroperitoneal fibrosis.

Female↗

PubMed1986

A comparison of cytoplasmic immunoglobulins in retroperitoneal fibrosis and abdominal aortic aneurysms.

Retroperitoneal fibrosis is associated with Riedel's thyroiditis, in which an unexpectedly high proportion of the plasma cells have been reported to contain IgA and lambda light chains. It has been suggested that retroperitoneal fibrosis and the inflammation and fibrosis in thick-walled abdominal aortic aneurysms are caused by a hypersensitivity reaction to antigens leaked from aortic atheroma. We examined cases of retroperitoneal fibrosis and aortic aneurysms in order to quantify the types of heavy and light chains in the plasma cells. A mean of 44% of the plasma cells contained IgA and 52% contained lambda light chain. These results provide further evidence of the pathological relationship between retroperitoneal fibrosis and Riedel's thyroiditis. It is suggested that a cross-reaction between antigens in mucosal surfaces and in the thyroid or retroperitoneum may be implicated, possibly involving vessel walls. Of the total plasma cells in the wall of the aortic aneurysms 24% contained IgA and 40% lambda, although there was a significant trend towards a higher proportion of IgA with increasing thickness of the wall. No definite support for a relationship between atheroma and retroperitoneal fibrosis is provided by this study.

Aorta, Abdominal↗

PubMed2002

Spondyloarthropathy and retroperitoneal fibrosis: a case report.

Retroperitoneal fibrosis is an uncommon inflammatory condition in which the retroperitoneal tissue is transformed into a tight sheet of fibrous tissue. It can occur in association with various diseases, including rheumatic conditions. We report a new case in a 66-year-old man with spondyloarthropathy. Nine similar cases have been reported. Axial manifestations predominated, and half the patients carried the HLA-B27 antigen. The relation between retroperitoneal fibrosis and spondyloarthropathy remains controversial.

Aged↗

PubMed1995

Retroperitoneal fibrosis treated with tamoxifen.

Retroperitoneal fibrosis, although histologically benign, has a malignant course due to encasement and compression of tubular retroperitoneal structures, particularly the urinary system. The etiology of this neoplastic process is unclear, but clinical symptoms result from fibroblast proliferation with excessive collagen deposition. Because of the diffuse nature of this disease, surgical resection is technically impossible, and past medical therapies have been inconsistent. Tamoxifen has been shown to be effective in the treatment of desmoid tumors, and we present a patient with retroperitoneal fibrosis who was treated successfully with this anti-estrogen. Because of its minimal side effects and simplicity of dosing, tamoxifen may be a useful treatment in this disease.

Female↗

PubMed2000

[Our experience in the treatment of retroperitoneal fibrosis].

To discuss retroperitoneal fibrosis (RPF) etiopathogenesis and to report on our experience in the treatment of the disease. From 1977 to 1998 26 RPF patients, 15 idiopathic (I group) and 11 secondary (II group), entered our clinic. Vascular risk factors of the I group were cigarette smoking (73.3%) and arterial hypertension (46.6%). Etiologic factors of the II group were aorta abdominal aneurysm (four cases), radiation therapy for female genital tract cancer (four case), aorto-bifemoral bypass for aorta aneurysm (two cases), retroperitoneal non-Hodgkin lymphoma (one case). Treatment performed for idiopathic disease was medical in eight cases (alone in two, with endourologic measures in six) using corticosteroids in five and tamoxifen in three; only endourological in three and surgical in four (nephrectomy in two patients, ureterolysis and ureteroneocystostomy in one, pyeloureterolysis in one) with perioperative corticosteroid treatment in three cases. Treatments performed in patients with secondary disease were endourologic in five surgical in three (ureteroureterostomy, ureteroneocystostomy, pyeloureterolysis and pyeloreduction), medical with corticosteroids in two; one patient affected by perianeurysmatic fibrosis did not require any treatment because of disease's spontaneous recovery. Medical treatment induced symptom remission and plaque reduction in all patients. Surgery determined complete recovery in all patients except for one in whom the disease relapsed with controlateral urinary tract involvement. Medical RPF treatment for idiopathic or secondary disease is effective in the first stage. Disease management with tamoxifen is easy to perform, safe and effective. In the steady state of RPF the best results are obtained by surgery and perioperative glucocorticoid therapy reduced significantly fibrosis' relapse.

Adult↗

PubMed1991

The response of retroperitoneal fibrosis to tamoxifen.

Although retroperitoneal fibrosis is uncommon and histologically benign, it is a progressive and potentially fatal tumor. As the fibroblasts proliferate, they encase and may obstruct important retroperitoneal structures. Medical therapy in the past has been ineffectual, and since the tumor usually cannot be resected, surgery consists of lysis or bypass of the involved structures. Tamoxifen is effective in the treatment of desmoid tumors, and we report its use in two patients with retroperitoneal fibrosis with excellent results. The simplicity and safety of this treatment make tamoxifen an attractive choice of therapy.

Female↗

PubMed1997

[A case of retroperitoneal fibrosis with spontaneous regression].

Retroperitoneal fibrosis has an unknown pathogenesis. Medical treatment should be preferred to surgery. However, surgical treatment is required when an ureteral involvement is present. A case of retroperitoneal fibrosis with spontaneous regression is reported. Current knowledge on pathogenesis, diagnosis and preferred treatment is outlined.

Aged↗

PubMed2003

Interstitial nephritis associated with perianeurysmal retroperitoneal fibrosis.

Renal failure in retroperitoneal fibrosis is usually secondary to ureteric obstruction. We report a patient whose renal failure did not improve following relief of obstruction. Renal biopsy showed an interstitial nephritis, and function improved with steroid therapy. We suggest that renal biopsy should be considered in cases of renal failure associated with retroperitoneal fibrosis that do not respond to the relief of ureteric obstruction.

Aged↗

PubMed2002

Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration.

PURPOSE: Idiopathic retroperitoneal fibrosis is an uncommon disease of unknown etiology that may involve the ureters and other retroperitoneal structures. Surgical ureterolysis as well as medical treatment with steroids have been used to treat these patients. However, there is no agreement as to the dose and duration of steroid. We adopted a regimen of long-term steroid use in this prospective study. MATERIALS AND METHODS: We treated 12 patients with idiopathic retroperitoneal fibrosis with a regimen of steroids during a 10-year period. Tissue diagnosis was established by biopsy of all lesions and ureteral obstruction was managed with insertion of a nephrostomy tube or a ureteral stent. The initial dose of prednisolone was 60 mg. on alternate days for 2 months and was tapered during the following 2 months to a daily dose of 5 mg. The total duration of prednisolone use was 2 years. RESULTS: Of the 12 patients 11 who completed this treatment regimen have been followed for a duration of 26 to 132 months (median 63.1) after discontinuation of treatment. Good response in the form of relief of symptoms and regression of the mass occurred in 9 cases and there were 2 failures. In 1 case the retroperitoneal mass did not regress and surgical ureterolysis was required. In the 2nd case symptoms recurred after discontinuation of steroid and a further small dose of steroids was required. Function deteriorated in 1 of 19 functioning renal units. No steroid related serious side effects developed. CONCLUSIONS: This regimen of steroid may be used as the primary mode of treatment for the majority of patients with idiopathic retroperitoneal fibrosis with minimal complications. Patients with idiopathic retroperitoneal fibrosis should be followed periodically for the rest of their lives.

Adult↗