AUTHOR AND EDITOR INFORMATION

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• Medication
• Follow-up
• Miscellaneous
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INTRODUCTION

Section 2 of 11  

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Background

Retroperitoneal fibrosis (RPF) is characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae. This fibrosis leads to entrapment and obstruction of retroperitoneal structures, notably the ureters. In most cases, the etiology is unknown. However, its occasional association with autoimmune diseases and its response to corticosteroids and immunosuppressive therapy suggest it is probably immunologically mediated (Katz, 1977; Carton, 1969). Approximately 8% of cases may be associated with metastatic malignancy (Lepor, 1979).

The symptoms and signs associated with retroperitoneal fibrosis are nonspecific, and diagnosis requires a high degree of suspicion. Although a definitive diagnosis can only be made based on biopsy findings, intravenous urography may provide further support for the diagnosis of retroperitoneal fibrosis, particularly if the classic features are present. CT scanning or MRI is essential for evaluating the extent of the disease process.

The aims in management of retroperitoneal fibrosis include preserving renal function, reducing the morbidity, and suppressing inflammatory processes. Although most cases are idiopathic, precipitating causes should be excluded, particularly malignancy. Surgical ureterolysis has been the preferred primary mode of treatment because it allows biopsy specimens to be obtained while ureteral obstruction is relieved. Recently, the knowledge of a possible autoallergic etiology has prompted the use of corticosteroids and cytotoxic drugs.

Pathophysiology

Anatomy of the retroperitoneum

The retroperitoneal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the transversalis fascia, and superiorly by the diaphragm. Inferiorly, it extends to the level of pelvic brim. The anterior and posterior layers of renal fascia (Gerota fascia) subdivide the retroperitoneal space on either side of the spine into 3 compartments. The posterior space contains the pararenal fat. The intermediate space contains the kidney, the adrenal gland, and the perirenal fat. The anterior space is more extensive.

The anterior pararenal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the anterior layer of renal fascia, and laterally by the lateral conal fascia. The anterior pararenal space contains the extraperitoneal portions of the ascending and descending colon, the duodenum, and the pancreas. The anterior pararenal space is continuous across the midline; however, collections of fluid tend to remain ipsilateral to the site of origin. Medially, the anterior layer of renal fascia blends with the connective tissue around the aorta and the inferior vena cava. The posterior layer fuses with the psoas fascia. Laterally, both layers merge to form lateral conal fascia.

Pathogenesis

Although the exact pathogenesis of retroperitoneal fibrosis has not been definitively described, good evidence supports the suggestion that it develops as an immunologic response to antigens within atherosclerotic plaques. Autopsy and CT studies have shown that fibrosis often begins around a severely atherosclerotic aorta. Thinning or breaching of the media may allow insoluble lipids, such as ceroid, an oxidized low-density lipoprotein, to leak into periaortic tissue, stimulating an immunologic reaction. This theory is supported by the presence of circulating anticeroid antibodies and the finding of ceroid-containing macrophages in nearby lymph nodes.

For this reason, Mitchinson suggested in a 1984 report that the condition should be termed chronic periaortitis. The frequent association of retroperitoneal fibrosis with aortic aneurysm and the regression of fibrosis reported following aneurysm repair further supports this theory (Pistolese, 2002). However, the occurrence of retroperitoneal fibrosis in children and those without an aneurysm suggests that other factors must be involved.

In some cases, an immune reaction to an external agent may initiate fibrosis. Drugs such as beta-blockers, methysergide, and methyldopa have been implicated, possibly by acting as haptens, leading to a hypersensitivity or autoimmune reaction (Higgins, 1990). The fibrous reaction associated with carcinoid tumor is believed to be the result of circulating serotonin or its metabolites. Methysergide is a strong serotonin antagonist, and the rebound release of serotonin following prolonged intake may be an alternate mechanism in this case. The association of retroperitoneal fibrosis with other connective tissue diseases and reported familial occurrences suggest genetic factors may also play a role (Hatsiopoulou, 2001). The human leukocyte antigen (HLA)–B27 cell marker has been demonstrated in several patients with retroperitoneal fibrosis (Astudillo, 1999).

Ureteral obstruction in retroperitoneal fibrosis often appears minimal despite severe renal failure. This suggests that the obstruction relates to impairment of normal ureteric peristalsis by fibrotic tissue rather than to mechanical obstruction.

Frequency

International

Retroperitoneal fibrosis is a relatively uncommon disease. The estimated annual incidence varies from 1 per 200,000-500,000 population (Higgins, 1988; Kottra, 1996).

Mortality/Morbidity

Because of the nonspecific nature of the symptoms, the diagnosis of retroperitoneal fibrosis is often delayed. This may lead to progressive loss of renal function.

Envelopment of the inferior vena cava and lymphatics may result in compression or thrombosis and leads to lower limb edema. In addition, involvement of gonadal vessels may cause scrotal edema. Occasionally, the duodenum, biliary tract, pancreas, large bowel, and mesentery may be involved (Inaraja, 1986; Hulnick, 1988).

Malignant retroperitoneal fibrosis is associated with poor prognosis, and most patients have an average survival of approximately 3-6 months (Arrivé, 1989). Idiopathic retroperitoneal fibrosis has a good prognosis, with little effect on long-term morbidity or mortality.

Race

No difference between ethnic groups has been observed.

Sex

Retroperitoneal fibrosis occurs twice as frequently in males as in females.

Age

The peak incidence is in people aged 40-60 years (Lepor, 1979; McDougal, 1991). Childhood presentation is extremely rare. To date, approximately 33 cases in children younger than 18 years have been reported (Dedeoglu, 2001).

CLINICAL

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History

The symptoms of retroperitoneal fibrosis are vague and nonspecific. Duration of symptoms prior to diagnosis is approximately 6-12 months (Buff, 1989; Gilkeson, 1996). Retroperitoneal fibrosis progresses through 2 clinical stages: (1) an early phase, which relates to the onset of inflammatory process, and (2) a late phase.

• The most common symptom (92% of cases) is a dull, poorly localized, noncolicky pain in the flank, back, scrotum, or lower abdomen (Koep, 1977). Other symptoms include fever, lower extremity edema, phlebitis, and deep venous thrombosis. In the pediatric age group, pain may be referred to the ipsilateral hip or gluteal region, resulting in resistance to hip extension (Birnberg, 1982).
• Other uncommon presentations include weight loss, nausea, vomiting, anorexia, and malaise.
• Rare manifestations reported in the literature include Raynaud phenomenon, ureteric colic, hematuria, claudication, and urinary frequency. Retroperitoneal fibrosis can be associated with Crohn disease, ulcerative colitis, and sclerosing cholangitis.

Physical

• Approximately half of the patients with retroperitoneal fibrosis have hypertension, which is probably the result of many factors. An increase in renin release secondary to obstructive uropathy may lead to hypertension. In some patients, volume-dependent hypertension may develop because of obstructive nephropathy.
• Occasionally, an abdominal mass, fever, or lower limb edema may be present.
• Obstruction of the ureters with a varying degree of renal insufficiency is the earliest and most common organ involvement.
• Uncommon physical findings due to late complications include the following:
• • Peripheral edema or thrombosis
  • Ascites
  • Hydrocele
  • Jaundice
  • Small or large bowel obstruction
  • Spinal cord compression

Causes

Sixty to 70% of cases of retroperitoneal fibrosis are idiopathic (Buff, 1989; van Bommel, 1991). However, a number of etiologic factors are known.

• Etiologic factors associated with retroperitoneal fibrosis are as follows:
• • Malignancy
  • Inflammatory periaortitis (severe atherosclerosis)
  • Chronic retroperitoneal inflammation
  • Retroperitoneal trauma
  • Autoimmune disease
  • Irradiation
  • Certain drugs (eg, beta-blockers, methysergide, methyldopa)
• In approximately 8% of patients, retroperitoneal fibrosis is associated with malignancy. This association is observed with lymphomas, sarcomas, and many carcinomas, including carcinomas of the breast, lung, stomach, colon, bladder, prostate, and cervix (Thomas, 1973). Metastatic tumor cells within the retroperitoneum cause an exuberant desmoplastic response. Malignant retroperitoneal fibrosis is radiologically distinct from retroperitoneal lymph node metastasis in that it typically displaces the ureter medically.
• Retroperitoneal fibrosis has been noted to be associated with connective tissue disease both in children and adults (Zabetakis, 1979; Izzedine, 2002).
• Immune-mediated connective tissue diseases in retroperitoneal fibrosis include the following:
• • Ankylosing spondylitis
  • Systemic lupus erythematosus
  • Scleroderma
  • Systemic vasculitis
  • • Wegener granulomatosis
    • Polyarteritis nodosa
    • Raynaud disease
  • Rheumatoid arthritis
  • Riedel thyroiditis
  • Immune complex membranous glomerulonephritis

DIFFERENTIALS

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Other Problems to be Considered

In the evaluation of patients with presumed idiopathic retroperitoneal fibrosis, exclude secondary retroperitoneal fibrosis due to malignancy, infection, retroperitoneal injury, or drugs.

WORKUP

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Lab Studies

• Blood
• • An elevated erythrocyte sedimentation rate (ESR)
  • Raised urea and creatinine levels (50-75%)
  • Normocytic normochromic anemia
  • Raised C-reactive protein
  • Polyclonal hypergammaglobulinemia
  • Alkaline phosphatase (has also been reported as a marker [Barrison, 1988])
• Urinalysis
• • Results are usually normal.
  • Rarely, microscopic hematuria or pyuria is observed.

Imaging Studies

• Plain radiography
• • Nonspecific findings are usually due to the late complications.
  • Plain abdominal radiography may show obliteration of the psoas shadow and an enlarged renal outline due to hydronephrosis. Features of ankylosing spondylitis or metastasis may also be visible.
  • Chest radiography may demonstrate pulmonary edema or fibrosis. Mediastinal widening may result from a soft tissue mass associated with mediastinal fibrosis.
• Intravenous urography
• • The classic triad includes delay of contrast material with unilateral (20%) or bilateral (68%) hydronephrosis, medial deviation of the middle third of the ureters (see Image 1), and tapering of the ureter at the level of L4/L5 vertebrae (Koep, 1977; Webb, 1967). Up to 18-20% of control subjects may show this triad (Saldino, 1972).
  • Medial deviation of the ureter may occur because of retroperitoneal neoplasm, aneurysm, and bladder diverticulum.
• Retrograde pyelography
• • Retrograde pyelography may show similar features as noted above. In addition, it may demonstrate poor distensibility of the ureters (see Images 2-3).
  • Retrograde pyelography delineates the pelvic calyceal anatomy and is usually performed prior to insertion of the stent to decompress the kidneys.
  • Interestingly, very little resistance is encountered during ureteric catheterization despite the extensive extrinsic fibrosis.
• Lymphangiography
• • The retroperitoneal lymphatics are delicate and fine structures; therefore, they are more easily compressed by retroperitoneal fibrosis than by the adjacent blood vessels and ureters. Thus, lymphatic obstruction should precede ureteric compression.
  • Lymphangiography may show obstruction of lymphatic flow at L3/L4 level, opacification of collateral channels, nonvisualization of lymphatics above the L4 vertebra, and delay in passage of contrast through the iliac and para-aortic lymphatics (Clouse, 1964).
• Ultrasonography
• • Ultrasonography is a simple noninvasive modality used to assess response to therapy.
  • On a sonogram, retroperitoneal fibrosis appears as a retroperitoneal, extensive, well-defined, hypoechoic mass centered over the sacral promontory.
  • The degree of hydronephrosis and hydroureter may vary.
  • Doppler sonography has no role in differentiating benign from malignant retroperitoneal fibrosis (Rubenstein, 1986).
• CT scanning
• • CT scanning is the most frequently used imaging method for diagnosis and follow-up of patients with retroperitoneal fibrosis.
  • On unenhanced CT scans, retroperitoneal fibrosis appears as a plaque that is isodense with muscle and that envelops the aorta and inferior vena cava between the renal hila and sacral promontory and usually extends laterally to incorporate the ureters. Obliteration of the fat plane between the mass and the psoas muscle may be observed (see Images 4-5).
  • Retroperitoneal hemorrhage, primary retroperitoneal sarcoma, metastatic deposits to the retroperitoneum, and retroperitoneal amyloidosis may show similar findings on CT scans. CT scan features that suggest malignant pathology include lateral displacement of the ureter, anterior displacement of the aorta, local bone destruction, and a large bulky lesion.
  • Elevation of the aorta from the spine is uncommon in benign retroperitoneal fibrosis. If present, it may be a sign of malignancy (Amis, 1991).
  • After contrast injection, the plaque may show a variable degree of enhancement, depending on the stage of the disease. Enhancement is usually significant in the early active vascular stage. On the other hand, enhancement is poor in the late vascular stage.
• Magnetic resonance imaging
• • Both benign and malignant retroperitoneal fibrosis have low-to-intermediate signal density on T1-weighted images and density on T2-weighted images that varies based on the disease stage. During the early stage, signal density is high because of high fluid content and hypercellularity. In contrast, late-stage disease has low T2 signal intensity as a result of avascular acellular fibrosis and decreased fluid content (Barker, 1995). Steroid therapy may lead to similar changes because of decreased tissue edema.
  • Inhomogeneity of signal intensity on T2-weighted images may suggest malignancy; however, differentiation between benign and malignant retroperitoneal fibrosis on MRI is difficult, and biopsy is usually required to confirm the diagnosis (Arrivé, 1989).
  • One study assessed delayed gadolinium enhancement ratios by comparing retroperitoneal fibrosis enhancement with that of psoas muscle. The dynamic enhancement ratio in acute disease significantly differed from the ratio in chronic disease. Dynamic gadolinium enhancement may be useful in differentiating newly diagnosed retroperitoneal fibrosis from treated chronic disease and may have a role in assessing disease activity, monitoring response to treatment, and detecting relapse (Burn, 2002).
  • MRI has some advantages over CT scanning, including multiplanar capability, independence of renal function, and absence of radiation.
• Nuclear medicine
• • Nuclear medicine has a very limited role.
  • In the acute phase, retroperitoneal fibrosis may take up gallium-67, possibly because of the binding of gallium-67 to lymphocytes (Liebman, 1983).
  • A small study of 2-deoxy-2-(F-18) fluoro-D-glucose (F-18DG) positron emission tomography scanning reported a low F-18DG uptake in retroperitoneal fibrosis and high uptake in malignant lymphoma (Kubota, 1992).
• Positron emission tomography
• • Positron emission tomography (PET) with 18F-fluorodeoxyglucose (18F-FDG) is a functional imaging modality used in oncology, but recent evidence suggests that it can be useful in the evaluation of various inflammatory diseases (Vaglio, 2005).
  • 18F-FDG identifies areas of high glucose metabolic activity. Because inflammatory cells have an increased glucose uptake, high levels of glucose metabolism are seen in a retroperitoneal mass associated with retroperitoneal fibrosis if inflammation is present.
  • No noninvasive method can reliably assess disease activity. However, 18F-FDG is useful for evaluating posttreatment disease. In addition, it can reveal other sites of disease (thyroid, thorax) and may help to identify the most appropriate sites for retroperitoneal biopsy.

Other Tests

• Biopsy
• • Open surgical biopsy is traditionally performed.
  • Tissue for histologic diagnosis can be obtained under CT or ultrasonographic guidance. In a 1999 publication, Dash et al described fine-needle aspiration for the diagnosis of retroperitoneal fibrosis, but most clinicians prefer a Tru-Cut needle biopsy.
  • In 1998, Pfammatter et al performed transcaval retroperitoneal core biopsies and suggested that the technique may have a role in patients at high operative risk, especially if the results from standard biopsies are inconclusive.

Histologic Findings

In 1948, Ormond described 2 histologic features in retroperitoneal fibrosis: an inflammatory early stage and a chronic stage. In the early stage, an inflammatory infiltrate contains macrophages, lymphocytes, plasma cells, and occasional eosinophils; neutrophils are generally absent. The macrophages are often lipid-laden and contain areas of perivascular lymphocytic infiltrate composed of T cells and B cells. Generally, tissue is highly vascular with numerous small blood vessels throughout. In the chronic stage, the tissue becomes avascular and acellular with scattered calcification and progresses to fibrous scarring (Mitchinson, 1970). Occasionally, surrounding structures are invaded by retroperitoneal fibrosis. Invasion of the large veins may cause fibrous thickening of the intima, resulting in complete occlusion. Periaortic lymphatics may be blocked within the mass. Submucosal edema and lymphocytic infiltration may be observed in the ureter.

Malignant retroperitoneal fibrosis demonstrates the presence of scattered nests of malignant cells within the inflammatory infiltrate. Hodgkin disease and sclerosing retroperitoneal lymphomas are the most challenging differential diagnoses for the pathologist to exclude. In 2002, Wu et al recommended the use of immunostains such as c-Kit, Leu-M1, Ki-1, LCA, and kappa and lambda light chain.

TREATMENT

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Medical Care

Optimal care of patients with retroperitoneal fibrosis requires an integrated approach of surgical and nonsurgical therapies. The aims of management are to preserve renal function, to prevent other organ involvement, to exclude malignancy, and to relieve symptoms. The literature reports no consensus on the appropriate management of patients with retroperitoneal fibrosis because no controlled therapeutic trials have been performed. Furthermore, successful outcome has occasionally been reported with conservative therapy (Adam, 1999; Kume, 2001). In 2002, Pistolese et al reported partial or complete regression of retroperitoneal fibrosis associated with inflammatory aortic aneurysm after surgery. The treatment of retroperitoneal fibrosis depends on the stage of the disease at diagnosis (see Image 6).

Empirical therapy includes corticosteroids, tamoxifen, and azathioprine; experimental therapy includes azathioprine, cyclophosphamide, mycophenolate-mofetil, cyclosporin, medroxyprogesterone acetate, and progesterone. Glucocorticoids and azathioprine are most useful in patients with signs of inflammation (eg, raised ESR and WBC count and positive ANA results).

• Corticosteroids
• • In 1958, Ross and Tinckler first reported the use of corticosteroids in the treatment of retroperitoneal fibrosis. The beneficial effect is thought to be due to anti-inflammatory action and the ability to inhibit fibrotic tissue maturation.
  • A pooled analysis of nonmalignant retroperitoneal fibrosis treated with steroids revealed a satisfactory outcome. In 2002, van Bommel analyzed 147 patients and noted good results in 122 patients (83%) and recurrence in 55 patients (16%). Most recurrences were noted within 12 months, and some responded to reintroduction of steroid. Despite their proven success, using steroids as a first-line therapy in retroperitoneal fibrosis remains controversial because many clinicians believe that multiple deep biopsies are still essential to exclude malignancy.
  • A standard protocol is prednisolone at 40-60 mg/d tapered to 10 mg/d within 2-3 months and discontinued after 12-24 months. Timely dose reductions and cessation are important because of the adverse effects associated with long-term steroid use.
  • In 1994, Harreby et al used methylprednisolone pulse therapy (MPPT) at 1 g/d IV for 3 days along with azathioprine or penicillamine. This therapy was used in 11 cases of retroperitoneal fibrosis with ureteric obstruction following initial insertion of ureteral stents. The treatment was successful in 7 patients but only moderately effective in 4 patients. The combination of glucocorticoid and azathioprine is most useful in patients with signs of inflammation (raised ESR, positive ANA results, positive PET findings).
  • Steroids can be used in combination with surgery. In one study, concomitant use of steroids with surgery reduced the rate of ureteric restenosis from 48% to 10% (Wagenknecht, 1981). However, response may vary, and an unacceptably high dose of steroid may be required to control retroperitoneal fibrosis.
  • Complications of corticosteroid treatment include obesity; Cushingoid features; striae; retarded growth; and increased susceptibility to infections, hypertension, osteoporosis, cataracts, peptic ulcer disease, and diabetes mellitus.
• Tamoxifen
• • In 1991, Clarke et al were the first to use tamoxifen, a nonsteroidal antiestrogen, in the treatment of retroperitoneal fibrosis.
  • Its mechanism of action is not entirely clear, and different hypotheses have been proposed. Tamoxifen increases the synthesis and secretion of transforming growth factor–beta (TGF-b), an inhibitory growth factor, by human fetal fibroblast in vitro. In retroperitoneal fibrosis, fibroblast and immune cells in the inflammatory mass may increase their secretion of TGF-b, which may then decrease the size of the fibrous plaque (Spillane, 1995). Other possible mechanisms of action include inhibition of protein kinase C, reduction of epidermal growth factor production, inhibition of calmodulin, and blockage of growth-promoting histaminelike receptor (Horgan, 1986; Loffeld, 1993).
  • Clinicians have reported successful treatment with tamoxifen. Various authors have used tamoxifen with a variable protocol (10-40 mg for 6 mo to 3 y).
  • Compared with steroids, the adverse effect profile of tamoxifen is low; thus, clinicians consider tamoxifen a reasonable treatment option. However, the adverse effects of tamoxifen, especially an increased risk of thromboembolism and ovarian cancer, should be carefully considered for each patient.
• Azathioprine: Azathioprine has been used when steroid therapy has failed and as a steroid-sparing drug. Cogan and Fastrez used a 6-week course of azathioprine (150 mg/d) in a patient whose condition recurred soon after prednisone treatment was discontinued. They observed a significant response with azathioprine. McDougal and MacDonell reported successful outcome in combination with prednisolone in a 14-year-old girl.
• Experimental therapy: More recently, immunosuppressive agents such as azathioprine, cyclophosphamide, mycophenolate-mofetil, methotrexate, and cyclosporin have been used to treat retroperitoneal fibrosis (McDougal, 1991; Marzano, 2001; Cogan, 1985; Scavalli, 1995; Kaipiainen-Seppanen, 1996).
• Medroxyprogesterone acetate: In vitro, medroxyprogesterone acetate inhibits fibroblastic proliferation. Use of progesterone and medroxyprogesterone acetate as an alternative treatment has been reported with successful outcome (Barnhill, 1987; Comini Andrada, 1985).

Surgical Care

• Temporizing maneuvers in the form of percutaneous nephrostomy or ureteral stenting are recommended in the presence of obstructive uropathy.
• Primary management of retroperitoneal fibrosis consists of open biopsy, ureterolysis, and lateral/intraperitoneal transposition or omental wrapping of the involved ureter (see Image 7).
• Open ureterolysis, although effective in 90% of patients, is associated with significant morbidity (60%) and mortality (9%) rates (Kerr, 1968; Miles, 1984).
• In the past 10 years, the use of laparoscopic surgery has expanded to include complex ablative and reconstructive procedures. Kavoussi first described laparoscopic ureterolysis for retroperitoneal fibrosis in 1992. Since then, a few authors have reported successful laparoscopic ureterolysis with a more rapid recovery and a shorter hospital stay. Although the success rate is no better than that of open ureterolysis, the laparoscopic technique has the advantage of reducing mean hospital stay, use of analgesia, convalescence period, and morbidity (Kavoussi, 1992; Matsuda, 1994; Fugita, 2002).
• More recently, with the advancement of technology, cases of endourologic treatment of retroperitoneal fibrosis via percutaneous balloon dilatation or endoscopic incision, dilatation, and permanent wall stent have been reported with varying results (Slavis, 2000).
• Long-term ureteral stenting is a reasonable approach in high-risk and elderly patients. Ureteral stenting may be placed on a long-term basis (months to years) in order to bypass ureteral obstruction. Short-term stenting (weeks to months) may be used as an adjunct to open surgical procedures.
• Other newer innovative surgical techniques have been described, such as ureterolysis and wrapping with Gore-Tex (GSM, WL Gore & Associates, Flagstaff, Ariz), excision of the ureter and reanastomosis, posterior preperitoneal flap, and renal autotransplantation.

Consultations

Patients with renal failure should be referred to a nephrologist early in the course of their disease and have continued nephrologic follow-up.

MEDICATION

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The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Drug Category: Glucocorticoids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli. Doses of up to 10 mg/d of prednisone or prednisolone are typically used, but some patients may require higher doses. Adverse events associated with long-term steroid use make dose reductions and cessation important in due course.

Drug Category: Immunosuppressives

Some agents function as antimetabolites to decrease DNA and RNA synthesis and are used to treat a number of autoimmune conditions. These agents can also be used in patients with inadequate response or excessive toxicity to corticosteroids.

Drug Category: Estrogen receptor antagonists

These agents bind to estrogen receptors, preventing stimulating effects of estrogen on nucleic acid synthesis.

FOLLOW-UP

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Further Inpatient Care

• After relief of long-standing obstruction, a physiologic diuresis is expected. This is usually a self-limiting process and can be managed conservatively with fluid and electrolyte replacement
• Urea diuresis is the most common. It is self-limiting, lasting 24-48 hours. Monitoring of fluid balance and electrolytes is required. Unless otherwise contraindicated, increased oral fluid intake should suffice.
• Sodium diuresis is the second most common postobstructive diuresis. It usually is self-limiting but may have a longer duration (>72 h). Monitor fluid balance and electrolytes more aggressively (ie, intake and output [I/O], central venous pressure [CVP], urine and serum electrolytes).
• Sonography may be used to assess hydronephrosis.

Further Outpatient Care

• Biochemical markers (eg, C-reactive protein, ESR, renal function) should be monitored every 4-8 weeks to assess the response.
• Radiologic assessment (eg, CT scanning, MRI) is performed every 3 months; once disease is stabilized, scanning can be repeated at 6 months.
• Recurrence of stenosis has been reported as late as 10 years; thus, long-term follow-up is necessary.
• Patients with renal failure should be referred to a nephrologist early in the course of their disease and have continued nephrologic follow-up.

Complications

• Renal failure
• Hypertension
• Scrotal edema
• Inferior vena cava thrombosis

Prognosis

• The natural history of retroperitoneal fibrosis has not been clearly established. However, outcome is generally good for nonmalignant retroperitoneal fibrosis (Baker, 2003).
• Prognosis is poor for malignant retroperitoneal fibrosis.

Patient Education

• Patients with renal failure should be educated about the importance of compliance with secondary preventative measures, natural disease progression, prescribed medications (highlighting their potential benefits and adverse effects), and diet.

MISCELLANEOUS

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Medical/Legal Pitfalls

• Because of the nonspecific clinical symptoms, the diagnosis is often overlooked in younger patients.
• Renal recovery is usually observed within the first 2 weeks. Checking these patients periodically is always better because some patients may regain renal function much later.

Special Concerns

• Tamoxifen studies have shown that women who take high doses of tamoxifen over a long period may have a slightly increased risk of developing uterine cancer.

MULTIMEDIA

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Media file 1:  Intravenous urogram shows medial deviation of the middle part of both ureters.
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Media file 2:  Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.
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Media file 3:  Retrograde pyelogram (same patient as in Image 2) demonstrates hydronephrosis.
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Media file 4:  Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
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Media file 5:  Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
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Media file 6:  Management algorithm of retroperitoneal fibrosis.
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Media file 7:  Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
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Media file 8:  Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
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Media file 9:  Abdominal radiograph demonstrates a wall stent on the right side.
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REFERENCES

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• Adam U, Mack D, Forstner R, et al. Conservative treatment of acute Ormond''s disease. Tech Urol. Mar 1999;5(1):54-6. [Medline].
• Albarran J. Retention renale par periureterite. Liberation externe de l'uretere. Association francaise d'urologie. 1905;9:511.
• Amis ES Jr. Retroperitoneal fibrosis. AJR Am J Roentgenol. Aug 1991;157(2):321-9. [Medline].
• Arrive L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology. Jul 1989;172(1):139-43. [Medline].
• Astudillo L, Alric L, Jamard B, Laroche M. [Retroperitoneal fibrosis in an HLA-B27-positive patient]. Rev Med Interne. Dec 1999;20(12):1149-50. [Medline].
• Baker LR. Auto-allergic periaortitis (idiopathic retroperitoneal fibrosis). BJU Int. Nov 2003;92(7):663-5. [Medline].
• Barker CD, Brown JJ. MR imaging of the retroperitoneum. Top Magn Reson Imaging. Spring 1995;7(2):102-11. [Medline].
• Barnhill D, Hoskins W, Burke T, et al. The treatment of retroperitoneal fibromatosis with medroxyprogesterone acetate. Obstet Gynecol. Sep 1987;70(3 Pt 2):502-4.
• Barrison IG, Walker JG, Jones C, Snell ME. Idiopathic retroperitoneal fibrosis--is serum alkaline phosphatase a marker of disease activity?. Postgrad Med J. Mar 1988;64(749):239-41.
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Article Last Updated: Nov 30, 2006