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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2015  |  Volume : 26  |  Issue : 4  |  Page : 816-822
Retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment and outcomes

Service de Médecine Interne, Hôpital Militaire Principal d'Instruction de Tunis, Tunis, Tunisia

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Date of Web Publication8-Jul-2015


Retroperitoneal fibrosis (RPF) is a rare disease and has a high risk of developing chronic kidney disease (CKD). This retrospective study was carried out with the objective to study the epidemiological, clinical and therapeutic characteristics of RPF and identify the risk factors associated with its progression to CKD. All 30 cases (24 males and five females) of RPF admitted from January 1985 to December 2013 in the Military Hospital of Tunis were included in this study. The mean age was 50.5 years. Presentation was with lower back pain, acute renal failure and inflammatory syndrome in 93%, 56% and 43% of the cases, respectively. Sixteen patients (54%) had a creatinine clearance <60 mL/min at the time of diagnosis. Erythrocyte sedimentation rate and C-reactive protein of >30 mm/h and 10 mg/L were observed in 56% and 53% of cases, respectively. The abdominal computed tomography scan showed ureterohydronephrosis in 63% of the cases. Classes I, II and III according to Scheel's radiological classification were found in, respectively, 16%, 13% and 70% of cases. Biopsy of RPF was performed in 20% of the cases, and all showed an inflammatory infiltrate without signs of vasculitis. RPF was idiopathic in 85% of the cases. Oral corticosteroid therapy was started for all patients. After a mean follow-up time of 53.2 months, an initial favorable response was noted in 76% of the cases. Fifty-three percent of the patients have presented one or more relapses during follow-up and 20% progressed to CKD. Most relapses were successfully treated by corticosteroids; only five patients had required additional immunosuppressive therapy. Two patients died. Elevated creatinine at diagnosis, high urea, clearance of creatinine lower than 60 mL/min and the use of ureteral stents were identified as risk factors for development of CKD.

How to cite this article:
Labidi J, Ariba YB, Chargui S, Bousetta N, Louzir B, Othmani S. Retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment and outcomes. Saudi J Kidney Dis Transpl 2015;26:816-22

How to cite this URL:
Labidi J, Ariba YB, Chargui S, Bousetta N, Louzir B, Othmani S. Retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment and outcomes. Saudi J Kidney Dis Transpl [serial online] 2015 [cited 2022 Nov 26];26:816-22. Available from: https://www.sjkdt.org/text.asp?2015/26/4/816/160226

   Introduction Top

Retroperitoneal fibrosis (RPF) is a rare disease characterized by inflammation and fibrosis surrounding the infrarenal aorta and other retroperitoneal structures. [1] RPF is divided into primary (idiopathic) and secondary disease. The pathogenesis is still poorly understood, but numerous theories have been discussed in previous reports. [2] The clinical presentation of RPF is usually insidious; the common symptoms are low back or abdominal pain and impaired general condition. Acute renal failure (ARF) is one of the initial manifestations, reported as 32% in a large series of the Mayo Clinic. [3] Treatments consisted of a combination of medications with or without surgical intervention. Relapse rate varies from one series to another. The disease prognosis is mainly related to renal functional outcome and complications of systemic corticosteroids. The aim of our study was to perform a descriptive study of the epidemiological, clinical, etiologic, therapeutic and evolutionary factors as well as an analytical study of the risk factors associated with development of renal failure in RPF.

   Patients and Methods Top

We conducted a retrospective study of all the cases of RPF admitted in the Internal Medicine Department of the Military Hospital, Tunis, between January 1985 and December 2013. RPF diagnosis was based on the following criteria:

  • Infiltration surrounding aorta, in renal and/or iliac vessels seen on an imaging study, either abdominal computed tomography (CT) or magnetic resonance imaging (MRI)
  • Histological evidence was not considered necessary for diagnosis.

Data collection was carried out from a pre-determined grille recording clinical, paraclinical and outcomes features.

We opted for the Scheel radiological classification to assess the extent of plaque fibrosis. [4]

  • Class I: Soft-tissue density surrounding the infrarenal aorta and/or iliac vessels
  • Class II: Soft-tissue density surrounding the infrarenal vena cava
  • Class III: Lateral extension of the inflammation/fibrosis with compression of one or both ureters
  • Class IV: Extension of fibrosis, which includes the renal hilum with compression of the renal artery and/or renal vein.

Medications including glucocorticoids or immunosuppressive therapy and urological interventions including placement of ureteral stents, percutaneous nephrostomy tubes, ureterolysis and vascular stenting or surgery were also recorded.

Laboratory follow-up included white blood cells (WBC), erythrocyte sedimentation rate, C-reactive protein (CRP) and serum creatinine levels. Radiologic follow-up included renal ultrasonography or computed tomography (CT) every six months. The existence of renal failure at diagnosis and at the end of treatment was defined by a serum creatinine clearance lower than 60 mL/min. Chronicity was defined by persistence beyond three months. Relapse was defined as either the reappearance of painful symptoms and/or the presence of one of the parameters of the biological inflammatory syndrome (BIS) associated with a worsening of imaging findings.

For statistical analyses, statistical software SPSS 19.0 was used. Quantitative data were expressed as mean ± standard deviations. Qualitative variables were expressed as percentages. A P-value of <0.05 was considered significant.

   Results Top

Of the 30 patients, 24 were men and six were women. The mean age at diagnosis of RPF was 50.5 years (range 32-77 years). The cardiovascular risk factors identified were smoking in 53%, hypertension in 13% and diabetes in 6% of patients. Lower back pain was the most responded mode of revelation, described by 93% of patients. ARF and BIS was seen in, respectively, 56% and 43% of the cases. The average time of onset of symptoms at the time of diagnosis was 15.4 weeks.

Clinically, a weight loss exceeding 3 kg weight and low-grade fever were observed in, respectively, 60% and 10% of the cases. Signs of venous compression were diagnosed by lower limb edema in 13% and hydrocele in 3% of the cases. Urine output was preserved in 97% of all cases.

The mean creatinine value at diagnosis was 296.7 μmol/L. The mean creatinine clearance at the time of diagnosis was 61.03 mL/min. More than half the population (54%) had a creatinine clearance at diagnosis <60 mL/min. Elevated inflammatory markers were found in 20 patients (66.7%) with ESR <0 mm/h and CRP above 10 mg/L noted, respectively, in 56% and 53%. Mean hemoglobin was 12.35 g/dL. Anemia was noted in 16%, and most often was of inflammatory origin. The mean leukocyte count was 8.953/mm 3 . Leukocytosis was noted in 20% of the cases.

Tests for ANA and ANCA were negative in all cases and the serum complement levels were within normal limits. Evaluation of immunoglobulin G4 (IgG4) autoimmune activity was carried out in one case, and it was normal.

All patients underwent abdominal CT scan, which showed ureterohydronephrosis in 63% of the cases [Figure 1], unilateral and bilateral, respectively, in 20% and 43% of the cases. Classes I, II and III were found in, respectively, 16%, 13% and 70% of the cases. Renal atrophy was observed in 16% of the cases. The clinical, biochemical and radiological presentations of the 30 patients with RPF are summarized in [Table 1].
Figure 1: Computed tomogram demonstrating retroperitoneal fibrosis.

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Table 1: Clinical, biological and radiological presentations of the 30 patients with RPF.

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Biopsy of the retroperitoneal lesions was performed in 20% of the cases, showing in all cases an inflammatory infiltrate without evidence of vasculitis. Two biopsies were performed surgically and four were performed by CT-guided punctures.

RPF was idiopathic in 85% of the cases and secondary in 15% of the cases, with abdominal trauma in two cases and abdominal surgery, tuberculosis and sclerotic pancreatitis in one case each.

The details of treatment carried out are given in [Table 2]. In the initial phase, 50% of our patients were treated with ureteral stenting. Oral glucocorticoids were started for all patients at a mean initial dose of 0.98 mg/kg per day. Twenty-nine patients received an initial dose of 1 mg/kg and one patient received an initial dose of 0.5 mg/kg/day. One case was also given anti-tuberculosis treatment.
Table 2: Type of medical and surgical therapy in 30 patients with retroperitoneal fibrosis.

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Over the entire follow-up, five patients (16.7%) received immunosuppressive treatment (methotrexate in four cases, mycophenolate mofetil in one case) along with low-dose corticosteroids.

All patients had follow-up for an average period of 53.2 months. Ten patients were followed for more than 48 months. Nine patients (30%) underwent a change of ureteral stents. Nephrostomy, ureterolysis and nephrectomy were required in, respectively, 3%, 6% and 3% of all cases

At the last follow-up, 18 patients (60%) were weaned from steroid therapy, while others received an average of 9.5 mg/day of corticosteroids. One patient died because of sepsis and another of undetermined cause. The duration of maintenance therapy was prolonged for an average time of 23.9 months.

Initial favorable response was noted in 76% of the cases. Fourteen patients (47%) never had a recurrence. More than half of the patients (53%) presented with one or more relapses during follow-up. Seventy-three percent of the patients maintained a serum creatinine clearance >60 mL/min and 20% progressed to chronic kidney disease (CKD).

In univariate analysis, the factors associated with development of CKD were high creatinine at diagnosis, high urea, creatinine clearance <60 mL/min and the use of ureteral stents [Table 3].
Table 3: Epidemiological, clinical, biological and therapeutic correlation depending on renal prognosis.

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   Discussion Top

Our study shows the importance of associating clinical, biological and radiological data with the diagnosis of RPF, therapeutic arsenal and the prognosis over time related to different treatment protocols. Our study has its strength from the long follow-up of our patients. The originality of this study was due to the fact that illustrates the evolution of RPF from an obstructive kidney disease usually handled by urologists to an inflammatory disease better managed by early medical care and the development of different therapeutic protocols with corticosteroids, methotrexate and/or MMF. However, a weak point to note is the small size of our study population, but still a respectable number considering the rarity of the RPF.

RPF is a rare disease, characterized by the presence of chronic inflammation and marked fibrosis of retroperitoneal tissue, which often entraps the ureters and/or other abdominal organs. The epidemiology of RPF is not well known, but its prevalence has been reported as 1.4/100,000 inhabitants, and its incidence varies from 0.1 to 1.3/100,000 person-years. [5],[6] The pathogenesis of the idiopathic disease is unclear. Recent evidence supports the hypothesis that the disease may be the result of an inflammatory state triggered by autoimmune responses. Possibilities include genetic components, environmental factors, immunologic process such as IgG4-bearing plasma cell deposition and local inflammatory complication of advanced atherosclerosis. [2] It occurs predominantly in men in their fifth and sixth decades of life, with a 3:1 male/female ratio and no ethnic predisposition. The mean age of our patients was 50.5 years which, is similar to that in previous reports. [7]

The most common symptom in our patients was pain (abdominal, flank or back), which was similar to other reports. Other frequent symptoms were fever, weight loss, generalized weakness and oliguria. Lower extremity edema was also a common symptom, reflecting the compression of the ilio-femoral veins by RPF. [2] Elevated inflammatory markers are often found. The value of these data must be recorded and used as a reference for monitoring and evolution of the disease under treatment. [8]

ARF is a condition that most often leads to the discovery of the disease. It is present in 94% of the cases, sometimes severe with anuria requiring dialysis. It can be progressive, with preserved urine output usually leading to chronic interstitial nephritis. [9] In the series of Kaaroud et al, [10] ARF was found in 73% of the patients. Renal failure at the time of diagnosis is found in a lesser number of cases in our series (56%) than in other studies. [11],[12] The lower incidence of renal failure at diagnosis and higher number of inflammatory syndrome in our series show the change in the approach to the disease from a late-presenting obstructive kidney disease usually managed by urologists to an inflammatory disease receiving earlier medical care.

Abdominal ultrasound reveals retroperitoneal fibrosis as a hypoechoic or isoechoic mass, which can involve the ureters and thus cause unilateral or bilateral hydronephrosis. CT and MRI are the most reliable modalities for diagnosis because they are non-invasive and provide a clear picture of the disease. [13] MRI imaging with dynamic enhancement analysis by Gladolinium was able to distinguish between patients with different response rates to medical treatment of IRF and may be useful in individualizing therapeutic decision-making. [14] Although histology remains the gold standard for positive and definitive diagnosis, biopsies are currently only used to eliminate specific secondary forms (neoplastic or infectious causes).

Two forms of the disorder have been described in the published literature. The idiopathic form of the disease accounts for more than two-thirds of the cases. [11] The most common causes of secondary RPF are medications like ergot derivatives, beta blockers, hydralazine and analgesics. Other causes of secondary RF include malignancies, infections, radiotherapy and surgery. [15]

Treatment of RPF depends on whether it is idiopathic or secondary. The goals of therapy are to relieve the obstruction, stop the progression of the fibrotic process and prevent disease relapse. Treatment modalities available include medical and surgical therapies. Glucocorticoids are considered the mainstay of therapy for RPF. If the disease does not adequately respond to steroid therapy alone, immunosuppressive agents can be used concurrently with steroids. Agents that have been used with apparent success in case reports and case series include azathioprine, methotrexate, mycophenolate mofetil cyclophosphamide and cyclosporine.

Tamoxifen showed no value in maintenance therapy in a clinical trial, but has been recently proposed by Vaglio et al as first-line treatment in an uncontrolled trial, especially if there is contraindication for corticosteroid therapy in patients without urinary tract dilatation. [16]

The ureterolysis was for decades the only therapeutic method of RPF. [17] Used alone, ureterolysis gives results, but recurrence is common. The absence of plaque regression after treatment occurs in 8% of cases. [18] Ureteral decompression by ureteral stents or nephrostomy tubes is recommended for definite cases of RPF with obstruction, with or without glucocorticoids is considered as standard treatment in recent years. [2]

Follow-up data were available for all the patients in this study. Therapeutic monitoring was based on the biochemistry and radiology in different series in the literature. The prognosis of FRP appears closely related to the etiology. In idiopathic forms, the prognosis is related to renal involvement. Under treatment, between 67% and 80% of patients do not develop renal sequelae. [19] No predictors have been identified for disease relapse. [13] The relapse rate after discontinuation of treatment ranges from <10% to 30%, lesser than our results because shortness of the follow-up period and the definition of relapse were not standardized.

Risk factors of CKD have rarely been evaluated in the literature. Gallais, in a recent study, identified the age of patients, the presence of diabetes and the initial value of creatinine as factors predicting CKD. The small numbers of this study make these findings not very powerful, but they remain logical, these being major factors in the development of CKD. [20]

These results need to be confirmed by a prospective multi-center study with a larger number of patients. The management and diagnostic and therapeutic monitoring of RPF are not standardized. Further studies are needed to determine optimal treatment, frequency of imaging needed to assess disease activity, required duration of therapy, predictors of response to therapy and long-term outcomes.

   References Top

Scheel PJ Jr, Sozio SM, Feeley N. Medical management of retroperitoneal fibrosis. Trans Am Clin Climatol Assoc 2012;123:283-90.  Back to cited text no. 1
Ha YJ, Jung SJ, Lee KH, Lee SW, Lee SK, Park YB. Retroperitoneal fibrosis in 27 Korean patients: Single center experience. J Korean Med Sci 2011;26:985-90.  Back to cited text no. 2
Kermani TA, Crowson CS, Achenbach SJ, Luthra HS. Idiopathic retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc 2011;86:297-303.  Back to cited text no. 3
Scheel PJ Jr, Feeley N. Retroperitoneal fibrosis: The clinical, laboratory, and radiographic presentation. Medicine (Baltimore) 2009;88:202-7.  Back to cited text no. 4
Uibu T, Oksa P, Auvinen A, et al. Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 2004;363:1422-6.  Back to cited text no. 5
van Bommel EF, Jansen I, Hendriksz TR, Aarnoudse AL. Idiopathic retroperitoneal fibrosis: Prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore) 2009;88:193-201.  Back to cited text no. 6
Katz R, Golijanin D, Pode D, Shapiro A. Primary and postoperative retroperitoneal fibrosis-experience with 18 cases. Urology 2002;60:780-3.  Back to cited text no. 7
Oshiro H, Ebihara Y, Serizawa H, et al. Idiopathic retroperitoneal fibrosis associated with immunohematological abnormalities. Am J Med 2005;118:782-6.  Back to cited text no. 8
Todd GJ, DeRose JJ Jr. Retroperitoneal approach for repair of inflammatory aortic aneurysms. Ann Vasc Surg 1995;9:525-34.  Back to cited text no. 9
Kaaroud H, Jeri E, Béji S, et al. Retroperitoneal fibrosis. Presse Med 2005;34:213-7.  Back to cited text no. 10
Koep L, Zuidema GD. The clinical significance of retroperitoneal fibrosis. Surgery 1977;81:250-7.  Back to cited text no. 11
Alexopoulos E, Memmos D, Bakatselos S, et al. Idiopathic retroperitoneal fibrosis: A longterm follow-up study. Eur Urol 1987;13:313-7.  Back to cited text no. 12
Ezimora A, Faulkner ML, Adebiyi O, Ogungbemile A, Marianna SV, Nzerue C. Retroperitoneal fibrosis: A rare cause of acute renal failure. Case Rep Nephrol 2012;2012: 645407.  Back to cited text no. 13
Brandt AS, Kamper L, Kukuk S, Piroth W, Haage P, Roth S. An aid to decision-making in therapy of retroperitoneal fibrosis: Dynamic enhancement analysis of gadolinium MRI. J Clin Med Res 2013;5:49-56.  Back to cited text no. 14
Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006;367:241-51.  Back to cited text no. 15
Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: An openlabel randomized controlled trial. Lancet 2011;378:338-46.  Back to cited text no. 16
De Luca S, Terrone C, Manassero A, Rocca Rossetti SA. Etiopathogenesis and treatment of idiopathic retroperitoneal fibrosis. Ann Urol 1998;38:153-9.  Back to cited text no. 17
Kardar AH, Kattan S, Lindstedt E, Hanash K. Steroid therapy for idiopathic retroperitoneal fibrosis: Dose and duration. J Urol 2002; 168:550-5.  Back to cited text no. 18
Lugosi M, Sacré K, Lidove O, et al. Long-term follow-up of a French cohort of retroperitoneal fibrosis. Rev Med Interne 2013;34:591-9.  Back to cited text no. 19
Gallais Sérézal I, Le Jeune S, Belenfant X, et al. Idiopathic retroperitoneal fibrosis: A multicentric retrospective study of 30 French cases and follow-up of the renal function. Rev Med Interne 2014;35:570-6.  Back to cited text no. 20

Correspondence Address:
Jannet Labidi
Service de Médecine Interne, Hôpital Militaire Principal d'Instruction de Tunis, Tunis
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.160226

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  [Figure 1]

  [Table 1], [Table 2], [Table 3]

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