eMedicine Specialties > Urology > Hydronephrosis and Ureter Disorders

Retroperitoneal Fibrosis

Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU, Consulting Urologist, Department of Urology, Pinderfields General Hospital, UK
Joby Taylor, MB, ChB, MRCS, Specialist Registrar in Urology, Yorkshire Deanery, UK; Anthony J Browning, MB, ChB, FRCS(Edin), Consultant Urological Surgeon, Associate Director of Medical Education, Mid Yorkshire NHS Trust, Pinderfields General Hospital; Training Program Director for Urology, Yorkshire and Humber Deanery, UK

Updated: Jun 9, 2009

Introduction

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.^1,2 Approximately 8% of cases are associated with metastatic malignancy.³

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.⁴ The frequent association of retroperitoneal fibrosis with aortic aneurysm and the regression of fibrosis reported following aneurysm repair further supports this theory.⁵ 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.⁶ 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.⁷ The human leukocyte antigen (HLA)–B27 cell marker has been demonstrated in several patients with retroperitoneal fibrosis.⁸

Recently, investigators have coined the term hyper–immunoglobulin G4 (hyper-IgG4) syndrome owing to raised levels of IgG4 and abundant IgG4-rich plasma cells in the inflammatory infiltrate.⁹ Pathogenetic features of idiopathic retroperitoneal fibrosis include the following:

• HLA-B27 or HLA-DRB1 03 haplotype
• High serum IgG4 concentration
• Raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level
• Positive serum autoantibodies
• Marked infiltration of IgG4-positive plasma cells

A 2004 case-control study increased that smoking and exposure to asbestos also increase the risk of retroperitoneal fibrosis.¹⁰

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.^11,12

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 are involved.^13,14

Malignant retroperitoneal fibrosis is associated with poor prognosis, and most patients have an average survival of approximately 3-6 months.¹⁵ Idiopathic retroperitoneal fibrosis carries a good prognosis, with little effect on long-term morbidity or mortality.

Race

Retroperitoneal fibrosis has no reported racial predilection.

Sex

Retroperitoneal fibrosis is twice as common in males as in females.

Age

The peak incidence of retroperitoneal fibrosis is in adults aged 40-60 years.^3,16 Childhood presentation is extremely rare. To date, approximately 33 cases in children younger than 18 years have been reported.¹⁷

Clinical

History

The symptoms of retroperitoneal fibrosis (RPF) are vague and nonspecific. Duration of symptoms prior to diagnosis is approximately 6-12 months.^18,19 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.²⁰ Other symptoms include fever, lower-extremity edema, phlebitis, and deep venous thrombosis. In children, pain may be referred to the ipsilateral hip or gluteal region, resulting in resistance to hip extension.²¹
• Other uncommon presentations include weight loss, nausea, vomiting, anorexia, and malaise.
• Rare manifestations of retroperitoneal fibrosis 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 individuals 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 result from obstructive nephropathy.
• Occasionally, an abdominal mass, fever, or lower limb edema is 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.^18,22 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.²³ 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 associated with connective tissue disease both in children and adults.^24,25
• 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

Differential Diagnoses

Other Problems to Be Considered

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

Workup

Laboratory Studies

• Blood
  • An elevated ESR
  • Raised urea and creatinine levels (50%-75%)
  • Normocytic normochromic anemia
  • Raised C-reactive protein level
  • Polyclonal hypergammaglobulinemia
  • Alkaline phosphatase (has also been reported as a marker²⁶ )
  • Antinuclear antibodies (ANA; present in 60% of cases)²⁷
• 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.^20,28 Up to 18%-20% of control subjects may show this triad.²⁹
    
    

    

    Intravenous urogram shows medial deviation of the middle part of both ureters.

    
    
  • Medial deviation of the ureter may result from retroperitoneal neoplasm, aneurysm, and bladder diverticulum.
• Retrograde pyelography
  • Retrograde pyelography may show features similar to those described above. In addition, it may demonstrate poor distensibility of the ureters (see Images 2-3).
    
    

    

    Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.

    
    
    
    

    

    Retrograde pyelogram (same patient as in Image 2) demonstrates hydronephrosis.

    
    
  • 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.³⁰
• 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 ultrasonography has no role in differentiating benign from malignant retroperitoneal fibrosis.³¹
• CT scanning
  • CT scanning is the most frequently used imaging method for diagnosis and follow-up of 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).
    
    

    

    Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.

    
    
    
    

    

    Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.

    
    
  • 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.³²
  • 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.³³ 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.¹⁵
  • One study assessed delayed gadolinium enhancement ratios by comparing retroperitoneal fibrosis enhancement with that of psoas muscle. The dynamic enhancement ratio in acute retroperitoneal fibrosis significantly differed from the ratio in chronic retroperitoneal fibrosis. 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.³⁴
  • 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.³⁵
  • A small study of 2-deoxy-2-(F-18) fluoro-D-glucose (F-18DG) positron emission tomography (PET) scanning reported a low F-18DG uptake in retroperitoneal fibrosis and high uptake in malignant lymphoma.³⁶
• Positron emission tomography
  • PET scanning 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.³⁷
  • 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
  • A wide spectrum of fibroinflammatory disorders can mimic retroperitoneal fibrosis on imaging. Management strategies are diverse and depend strongly on the histological diagnosis and extent of the disease. Histology and immunohistochemistry are required to confirm the diagnosis. The amount of tissue harvested via core needle biopsy may not be sufficient for the histological diagnosis. Therefore, open biopsy can ensure a definite histological diagnosis and is traditionally performed. However, it is associated with significant morbidity. Laparoscopic biopsy is safe, minimally invasive, cost-effective, and useful in making therapeutic decisions for retroperitoneal masses.
  • 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.³⁸
  • 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.³⁹

Histologic Findings

In 1948, Ormond described two histologic features in retroperitoneal fibrosis: an inflammatory early stage and a chronic stage.⁴⁰ 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.⁴¹ 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.

Corradi et al (2007) recently reported the presence of CD20 and CD3 cells, IgG4 plasma cells, and subtle vasculitic activity in idiopathic retroperitoneal fibrosis.⁴²

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.⁴³

Treatment

Medical Care

Optimal care in patients with retroperitoneal fibrosis (RPF) 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.^44,45 In 2002, Pistolese et al reported partial or complete regression of retroperitoneal fibrosis associated with inflammatory aortic aneurysm after surgery.⁵ The treatment of retroperitoneal fibrosis depends on the stage of the disease at diagnosis (see Image 6).

Management algorithm of retroperitoneal fibrosis.

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.⁴⁶ 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).⁴⁷
  • 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%.⁴⁸ 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 an 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.⁴⁹
  • 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.⁵⁰ 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.^51,52
  • 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.
• Mycophenolate mofetil
  • To block the proliferation of T cells and B cells, a combination of mycophenolate mofetil (MMF) and steroid appears to be a promising option.
  • Swartz et al (2008) reported their experience with high-dose corticosteroid and MMF in 21 patients. They used prednisone 60 mg or 120 mg qid for 3-6 months and tapered the dose upon clinical improvement. In addition, steroid-sparing therapy with MMF (1000 mg bid) was recommended, with discontinuation after 6-12 months of therapy.⁵³
  • In a recent study, Adler et al (2008) observed radiologic regression following therapy with MMF 2 g/d and prednisolone 1 mg/kg in 9 patients. Therapy led to removal of the ureteral catheter in 5 of 7 patients. Complications of MMF therapy included recurrent urinary infections and upper gastrointestinal disturbance.⁵⁴  
• 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.⁵⁵ McDougal and MacDonell reported successful outcome in combination with prednisolone in a 14-year-old girl.¹⁶
• Experimental therapy: More recently, immunosuppressive agents such as azathioprine, cyclophosphamide, MMF, methotrexate, and cyclosporin have been used to treat retroperitoneal fibrosis. Future steroid-sparing options could include anti-inflammatory drugs such as tumor necrosis factor-alpha (TNF-a) inhibitors and anti-CD20 drugs.^{16,56,55,57,58}
• 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.^59,60

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).
  
  

  

  Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.

  
  
• Open ureterolysis, although effective in 90% of patients, is associated with significant morbidity (60%) and mortality (9%) rates.^61,62
• 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 1992.⁶³ 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.^63,64,65
• 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.⁶⁶
• 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

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

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.

Prednisolone (Pediapred, Delta-Cortef, Articulose-50, AK-Pred)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Dosing

Adult

10-60 mg/d PO qd or divided bid/qid; generally, maintenance dose should be <10 mg/d; alternatively, may be administered IM, IV, or intra-articularly

Pediatric

Not established

Interactions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI ulceration

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, and infections may occur with glucocorticoid use; abrupt discontinuation may cause adrenal crisis

Prednisone (Deltasone, Meticorten, Orasone)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Dosing

Adult

10-60 mg/d PO qd or divided bid/qid; generally, maintenance dose should be <10 mg/d; alternatively, may be administered IM, IV, or intra-articularly

Pediatric

Not established

Interactions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI ulceration

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, and infections may occur with glucocorticoid use; abrupt discontinuation may cause adrenal crisis

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.

Mycophenolate (CellCept, Myfortic)

Inhibits purine synthesis and proliferation of human lymphocytes. Promising published case report of 3 patients with resistant disease treated with mycophenolate mofetil. Reduced toxicity makes this regimen an attractive alternative.

Dosing

Adult

1 g PO bid

Pediatric

Not established

Interactions

In combination with either acyclovir or ganciclovir may result in higher levels for both interacting drugs due to competition for renal tubular excretion; aluminum/magnesium present in some antacids, and cholestyramine containing products may decrease absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates and azathioprine may increase toxicity; may decrease levonorgestrel AUC; may decrease live virus vaccine immune response; when administered in combination with theophylline may increase free fraction levels of theophylline; may reduce blood levels hormones contained in oral contraceptives and could reduce effectiveness

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Increases risk for infection (monitor blood count); severe renal impairment (CrCl <25 mL/min) may have increased adverse effects due to increased free MPA; caution in active peptic ulcer disease; incidence of malignancies and lymphoma consistent with that reported for other immunosuppressants (0.9%); commonly causes constipation, nausea, diarrhea, urinary tract infection, and nasopharyngitis; rare reports include interstitial lung disorders, colitis, pancreatitis, intestinal perforation, GI hemorrhage, gastric ulcers, duodenal ulcers, and ileus; do not chew, crush, or cut Myfortic tab; women of childbearing potential must have a negative serum or urine pregnancy test and must be completed within one week of beginning MMF and must receive contraceptive counseling and use effective contraception; continue contraception for 6 wk following discontinuation of drug

Azathioprine (Imuran)

Imidazolyl derivative of 6-mercaptopurine. Many of biological effects are similar to those of parent compound. Both compounds are eliminated rapidly from blood and are oxidized or methylated in erythrocytes and liver. No azathioprine or mercaptopurine is detectable in urine 8 h after administration.
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. Mechanism whereby azathioprine affects autoimmune diseases unknown. Works primarily on T cells. Suppresses hypersensitivities of cell-mediated type and causes variable alterations in antibody production.
Immunosuppressive, delayed hypersensitivity, and cellular cytotoxicity tests are suppressed to a greater degree than antibody responses. Works very slowly; may require 6-12 mo of trial prior to effect. Up to 10% of patients may have an idiosyncratic reaction that disallows use. Do not allow WBC count to drop to less than 3000/mL or lymphocyte count to drop to less than 1000/mL.
Available in tab form for oral administration or in 100-mg vials for IV injection.

Dosing

Adult

2-3 mg/kg/d PO in single or divided dose
Starting dose: 1 mg/kg/d PO; increase depending on clinical and hematologic response and toxicity

Pediatric

Not established

Interactions

Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine

Contraindications

Documented hypersensitivity; systemic infections; severe cytopenias

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Increases risk of neoplasia; caution in liver disease and renal impairment; hematologic toxicities may occur; check TPMT level prior to therapy and monitor liver, renal, and hematologic function; pancreatitis rarely associated

Estrogen receptor antagonists

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

Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)

Nonsteroidal antiestrogen agent. Competitively binds to estrogen receptor, producing nuclear complex that decreases DNA synthesis and inhibits estrogen effects.

Dosing

Adult

10-20 mg PO bid

Pediatric

Not established

Interactions

May exacerbate hepatotoxic effects of allopurinol; may increase cyclosporine serum levels; increases anticoagulant effects of warfarin; aminoglutethimide reduces serum concentrations of tamoxifen; cyclophosphamide, methotrexate, and 5-FU increase thrombotic risk of tamoxifen

Contraindications

Documented hypersensitivity; breastfeeding

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in leukopenia, thrombocytopenia, and hyperlipidemia; decreased visual acuity, corneal changes, and retinopathy may occur with >1 y of use; may induce ovulation

Follow-up

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).
• Ultrasonography may be used to assess hydronephrosis.

Further Outpatient Care

• Biochemical markers (eg, CRP, 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 (RPF) has not been clearly established. However, the outcome of nonmalignant retroperitoneal fibrosis is generally good.⁶⁷
• The prognosis of malignant retroperitoneal fibrosis is poor.

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

Medicolegal Pitfalls

• Because of the nonspecific clinical symptoms, the diagnosis of retroperitoneal fibrosis (RPF) 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

Media file 1: Intravenous urogram shows medial deviation of the middle part of both ureters.

Media file 2: Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.

Media file 3: Retrograde pyelogram (same patient as in Image 2) demonstrates hydronephrosis.

Media file 4: Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.

Media file 5: Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.

Media file 6: Management algorithm of retroperitoneal fibrosis.

Media file 7: Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.

Media file 8: Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.

Media file 9: Abdominal radiograph demonstrates a wall stent on the right side.

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Keywords

retroperitoneal fibrosis, RPF, periureteric fibrosa, periureteric plastica, periureteric fascitis, perinephritis plastica, ceroid, Ormond's disease, Ormond's syndrome, Ormond disease, Gerota's fascitis, Gerota fascitis, peripyelitis plastica stenosans, sclerosing lipogranuloma, sclerosing retroperitonitis, ureteral obstruction, ureteral entrapment, periureteritis, sclerosing retroperitoneal granuloma, chronic periaortitis, hyper-IgG4 syndrome

Contributor Information and Disclosures

Author

Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU, Consulting Urologist, Department of Urology, Pinderfields General Hospital, UK
Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU is a member of the following medical societies: British Association of Urological Surgeons, British Medical Association, European Association of Urology, and International College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Joby Taylor, MB, ChB, MRCS, Specialist Registrar in Urology, Yorkshire Deanery, UK
Joby Taylor, MB, ChB, MRCS is a member of the following medical societies: British Medical Association and Royal College of Surgeons of Edinburgh
Disclosure: Nothing to disclose.

Anthony J Browning, MB, ChB, FRCS(Edin), Consultant Urological Surgeon, Associate Director of Medical Education, Mid Yorkshire NHS Trust, Pinderfields General Hospital; Training Program Director for Urology, Yorkshire and Humber Deanery, UK
Anthony J Browning, MB, ChB, FRCS(Edin) is a member of the following medical societies: British Association of Urological Surgeons, Endourological Society, and European Association of Urology
Disclosure: Nothing to disclose.

Medical Editor

Martha K Terris, MD, FACS, Professor, Department of Surgery, Medical College of Georgia
Martha K Terris, MD, FACS is a member of the following medical societies: American Cancer Society, American College of Surgeons, American Institute of Ultrasound in Medicine, American Urological Association, New York Academy of Sciences, and Society of University Urologists
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Martin I Resnick, MD †, Former Lester Persky Professor and Chair, Department of Urology, Former Professor, Department of Oncology, Case Western Reserve University School of Medicine
Martin I Resnick, MD † is a member of the following medical societies: American College of Surgeons, American Federation for Medical Research, American Institute of Ultrasound in Medicine, American Medical Association, American Society for Bone and Mineral Research, American Society for Reproductive Medicine, American Society of Andrology, American Surgical Association, American Urological Association, Association for Academic Surgery, Endocrine Society, National Kidney Foundation, Ohio Urological Society, and Pan American Medical Association
Disclosure: Nothing to disclose.

CME Editor

J Stuart Wolf Jr, MD, FACS, David A Bloom Professor of Urology, Director of Division of Minimally Invasive Urology, Department of Urology, University of Michigan
J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology
Disclosure: Terumo Corporation Consulting fee Consulting; Omeros Corporation Consulting fee Consulting

Chief Editor

Bradley Fields Schwartz, DO, FACS, Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine
Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists
Disclosure: Nothing to disclose.

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