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Table of Contents
ORIGINAL ARTICLE
Year : 2019  |  Volume : 30  |  Issue : 3  |  Page : 114-117

Retroperitoneal fibrosis: Challenge in diagnosis and treatment


1 Department of Urology, Taipei Veterans General Hospital, National Yang-Ming University, Taipei, Taiwan
2 Department of Urology, Taipei Veterans General Hospital; Department of Urology, School of Medicine; Shu-Tien Urological Institute, National Yang-Ming University, Taipei, Taiwan

Date of Submission28-Apr-2017
Date of Decision16-Nov-2018
Date of Acceptance21-Dec-2018
Date of Web Publication20-Jun-2019

Correspondence Address:
Tzu-Chun Wei
Department of Urology, Taipei Veterans General Hospital, Taipei; Department of Urology, School of Medicine, Shu-Tien Urological Institute, National Yang-Ming University, Taipei
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/UROS.UROS_51_18

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  Abstract 


Objective: Literature documented for the diagnosis and treatment of retroperitoneal fibrosis (RF) is still relatively limited and diverse, which makes it more challenging, especially in the Taiwanese population. This article was aimed to describe the clinical manifestations, laboratory results, diagnostic tool, and treatments in patients with RF from a single tertiary institute. Patients and Methods: The International Classification of Diseases, Ninth Revision code 594.3 was used to identify patients evaluated for RF between January 2005 and August 2015, at Taipei Veterans General Hospital. Medical records were reviewed, and clinical information was collected. RF was diagnosed on the basis of image findings, as well as clinical, pathological, and laboratory data. Different treatments were compared for their efficacy on the protection of renal function. Results: A total of 30 patients were included in the study. Twenty-three (76.7%) were male. The mean age at diagnosis was 65.9 ± 16.37. Mean body mass index was 31.5 ± 3.47. Biopsies were done in 13 patients (43.3%). Half of the patients had serum IgG4 test, and the mean value was 249.3 ± 205.1 mg/dL. Bilateral hydronephrosis was noted in 19 (63.3%) patients and was associated with poorer renal function than unilateral hydronephrosis at baseline. Most (93.3%) of the patients had improved renal function. However, statistically significant improvement was noted in patients with bilateral hydronephrosis instead of unilateral one. Compared with endoscopic ureteral stenting, surgical correction tended to have more renal functional protection, with significant serum creatinine (P = 0.006) and estimated glomerular flow rate (P = 0.035) change. Conclusion: RF in Taipei Veterans General Hospital was diagnosed through image, with the assistance of serum IgG4 and biopsy. There was a posttreatment improvement of renal function, especially in those with bilateral hydronephrosis. Surgical correction tended to be more beneficial than endoscopic procedure.

Keywords: Diagnosis, retroperitoneal fibrosis, treatment


How to cite this article:
Liu CH, Wei TC, Lin AT, Chang YH, Wu HH, Kuo JY, Chung HJ, Huang EY, Lin CC, Huang WJ. Retroperitoneal fibrosis: Challenge in diagnosis and treatment. Urol Sci 2019;30:114-7

How to cite this URL:
Liu CH, Wei TC, Lin AT, Chang YH, Wu HH, Kuo JY, Chung HJ, Huang EY, Lin CC, Huang WJ. Retroperitoneal fibrosis: Challenge in diagnosis and treatment. Urol Sci [serial online] 2019 [cited 2019 Dec 14];30:114-7. Available from: http://www.e-urol-sci.com/text.asp?2019/30/3/114/260784




  Introduction Top


Retroperitoneal fibrosis (RF) is a relatively uncommon disease involving several different specialties such as rheumatology, urology, and cardiovascularity.[1],[2] Idiopathic RF can only be defined if all identifiable causes including malignancy, medication, or chronic infection have already been excluded.[2],[3] The management choices are very diverse, including interventions of obstruction relief such as endoscopic ureteral stenting, percutaneous nephrostomy (PCN) drainage, ureterolysis, intestinal reconstruction, and medications of immunosuppressive/antifibrotic agents such as corticosteroids, mycophenolate mofetil, cyclosporine, cyclophosphamide, methotrexate, azathioprine, and chlorambucil.[1],[2],[3] All these reasons result into the difficulty on the diagnosis and treatment of RF.

On the other hand, if the clinical condition is not recognized or left untreated, progressive disease may lead to compression of retroperitoneal structures, notably the ureters, which may gradually lead to hydronephrosis in different aspects.[4] Concerning about the renal function protection, there has still not been a standard management.[5] Therefore, we aimed to compare the outcome of several choices of managements, as well as the clinical manifestation and diagnosis tools, through a retrospective study from one single tertiary institute.


  Patients and Methods Top


Using the International Classification of Diseases, Ninth Revision code 594.3 for RF, we identified all patients ever evaluated at Taipei Veterans General Hospital from January 2005 to August 2015. Medical records were reviewed to confirm the diagnosis. The diagnosis was based on the compatible image findings, including idiopathic hydronephrosis with or without retroperitoneal mass surrounding the aorta and/or adjacent structures. Laboratory data and biopsy reports were also evaluated.

Baseline measurement

Clinical data including age, sex, height, weight, body mass index (BMI), initial renal function, and serum IgG4 level were collected. Diagnostic tools were also clarified through different types of image study (computed tomography [CT] or magnetic resonance imaging [MRI] of the abdomen or pelvis). The laterality and severity of hydronephrosis were also evaluated. Pathological reports of different biopsy methods (CT guided or ultrasound guided) were also reviewed.

Treatment

All medical and surgical managements for RF were recorded. Medications included glucocorticoids and other immunomodulators such as mycophenolate mofetil, cyclosporine, cyclophosphamide, methotrexate, and azathioprine. Surgical interventions included endoscopic placement of ureteral stents, PCN tubes, ureterolysis, and ureteral reconstruction with ileum.

Follow-up and outcome

The outcomes were defined mainly by the change of renal function, including serum creatinine and estimated glomerular flow rate (eGFR). Different treatments were compared for their efficacy on the protection of renal function.

Statistical analyses

The continuous data were analyzed with Mann–Whitney U-test (independent) and Wilcoxon signed-rank test (paired), and categorical data were compared with Fisher's exact test and logistic regression. Statistical analyses were performed with the Statistical Package for the Social Sciences, SPSS 17.0 (SPSS, Inc., Chicago, IL, USA), and the significance level was set as two-tailed, with P < 0.05 considered statistically significant.


  Results Top


A total of 30 patients were included in the study. Twenty-three were male (76.7%) and 7 were female. The mean age at diagnosis was 65.9 ± 16.37, and the mean BMI was 31.5 ± 3.47 [Table 1]. Diagnoses were confirmed according to the CT (80%) and MRI (20%) [Figure 1]a and [Figure 1]b. All patients had idiopathic hydronephrosis, and bilateral hydronephrosis was noted in 19 (63.3%). Biopsies were performed in 13 patients (43.3%). All biopsies were done through CT-guided method and reported as fibrosis and/or chronic inflammation without malignancy. Half of the patients had serum IgG4 test, and the mean value was 249.3 ± 205.1 mg/dL [Table 2].
Table 1: Patient characteristics (n=30)

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Figure 1: (a and b) This 94-year-old man had bilateral hydronephrosis related to an ill-defined soft-tissue mass at the para-aortic region with encasement of bilateral middle third of ureter. Biopsy revealed chronic inflammation and fibrosis

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Table 2: Image type, hydronephrosis laterality, IgG4 level, and biopsy

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One patient with CT-guided biopsy did not receive any further management and was lost to follow-up. Corticosteroids were initiated in five patients, and two of them also received immunomodulators. Medication alone worked in two of the five patients, but ureteral stenting was still needed among the rest three. The other surgical interventions were listed in [Table 3], including 17 ureteral stenting (10 by cystoscopy, 5 through ureteroscopy, and 2 through PCN antegradely), 4 ileal reconstruction, 2 ureterolysis, and 1 continuous PCN drainage.
Table 3: Treatment with medication and operation

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Bilateral hydronephrosis was associated with poorer renal function than unilateral hydronephrosis at baseline, with the mean serum creatinine level of 2.84 ± 2.19 versus 1.49 ± 0.56 mg/dL, P = 0.028. Most (93.3%) of the patients had numerically but no statistically improved renal function, with creatinine level of 2.28 ± 1.85 versus 1.60 ± 1.19 mg/dL, P = 0.056, and eGFR 42.3 ± 31.1 versus 55.1 ± 33.8, P = 0.134. Creatinine levels became within normal (<1.5 mg/dL) limit at the latest ambulatory visit in 16 patients (53.3%).

Hydronephrosis was also improved after ureteral procedure [Figure 2]a,[Figure 2]b,[Figure 2]c. However, statistically significant improvement was noted in patients with bilateral hydronephrosis (creatinine level of 2.84 ± 2.19 versus 1.42 ± 0.58 mg/dL, P = 0.017, and eGFR 37.3 ± 23.4 versus 54.4 ± 25.4, P = 0.043) instead of unilateral hydronephrosis (creatinine level of 1.98 ± 1.85 versus 1.49 ± 0.56 mg/dL, P = 0.414, and eGFR 50.0 ± 37.6 versus 56.2 ± 43.5, P = 0.741) [Table 4]. Compared with endoscopic ureteral stenting, surgical correction such as ileal reconstruction or ureterolysis tended to have more renal functional protection, with the creatinine change of 3.29 ± 1.36 versus 0.77 ± 0.18 mg/dL, P = 0.006, and eGFR change of 32.0 ± 6.28 versus 13.7 ± 1.38, P = 0.035 [Table 5].
Figure 2: (a-c) This 65-year-old man had elevated serum creatinine up to 6.2 mg/dL. Computed tomography scan showed a retroperitoneal lesion encasing abdominal aorta and inferior vena cava with extension to bilateral psoas muscle and compressed bilateral ureter. Biopsy revealed chronic inflammation and fibrosis. Bilateral hydronephrosis was improved after double J stenting

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Table 4: Comparison of Cr and estimated glomerular flow rate between baseline and posttreatment

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Table 5: Renal function improvement between surgical correction and endoscopic stenting

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A 65-year-old man had elevated serum creatinine up to 6.2 mg/dL. CT scan showed a retroperitoneal lesion encasing abdominal aorta and inferior vena cava with extension to bilateral psoas muscle and compressed bilateral ureter. Biopsy revealed chronic inflammation and fibrosis. Bilateral hydronephrosis was improved after double J stenting.


  Discussion Top


In our study, diagnoses were mainly established by image finding from CT or MRI. Not all patients with the diagnosis of RF would receive biopsy or serum IgG4 test. However, the pathological finding of fibrosis tissue and elevated serum IgG4 may contribute to the confirmation of RF diagnosis.[5] Not all patients would have bilateral hydronephrosis but those who did tend to have a poorer renal function at baseline. Patients undergoing ureteral procedures would have more improvement on renal function, regardless of ureteral stenting or ureterolysis or ileal reconstruction. Compared with endoscopic procedure, surgical correction tended to result in better renal function protection. However, patients receiving ureterolysis or ileal reconstruction were those who had higher baseline serum creatinine.

Recent review showed that biopsy remains the most reliable diagnostic tool for RF.[1]

The most common treatment is corticosteroid therapy alone or in combination with other immunosuppressants,[2] whereas surgical treatment is reserved for severe cases.[3] Indwelling ureteral stents represent the most common procedure for renal drainage, but their efficacy is questionable.[3],[4],[6] In our study, medical treatment was not as prevalent as ureteral stenting, possibly due to the fear from adverse events of steroid and immunosuppressants in the Taiwanese population. The similar treatment distribution was also found in another retrospective study from Mainland China.[3]

Up to the present, the largest database in Mayo Clinic identified 185 patients diagnosed with RF also revealed male predominance as in our study.[2] However, the mean age was older here in Taiwan, compared to the Western and Mainland Chinese population (65.9 ± 16.37 vs. 57.6 ± 11.82 and 56.7 ± 14.43). Zhou et al. reported the elevation of serum IgG4, in 9 of 22 patients (40.9%),[3] which was similar with our study. However, serum IgG4 and biopsy were not necessary but contributory diagnostic tools for RF.[5]

Our research was limited due to few patient numbers and retrospective analysis. Besides, we would lack for complete clinical data such as erythrocyte sedimentation rate or C-reactive protein, which were indicated to be distinguishable.[2] Still, we demonstrated the diagnostic tools and therapeutic managements to patients whoever suspicious of RF in the clinical practice, as well as the improvement of renal function through different modalities.


  Conclusion Top


Idiopathic RF in Taipei Veterans General Hospital was diagnosed through image findings, with the assistance of laboratory results such as serum IgG4 and the pathological report of biopsy. Medical treatment was less preferred and prevalent. Bilateral hydronephrosis was associated with more renal function impairment. However, statistically significant improvement was noted in patients with bilateral hydronephrosis instead of unilateral one. There was an improvement of renal function in patients with either endoscopic or surgical intervention (double J stent, ileum reconstruction, and ureterolysis), but surgical correction tended to be more beneficial than endoscopic procedure, especially for those with worse renal function at baseline.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Cristian S, Cristian M, Cristian P, Constantin G, Savu C, Huri E, et al. Management of idiopathic retroperitoneal fibrosis from the urologist's perspective. Ther Adv Urol 2015;7:85-99.  Back to cited text no. 1
    
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. 2
    
3.
Zhou HJ, Yan Y, Zhou B, Lan TF, Wang XY, Li CS, et al. Retroperitoneal fibrosis: A retrospective clinical data analysis of 30 patients in a 10-year period. Chin Med J (Engl) 2015;128:804-10.  Back to cited text no. 3
    
4.
Jadhav KK, Kumar V, Punatar CB, Joshi VS, Sagade SN. Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective. Investig Clin Urol 2017;58:371-7.  Back to cited text no. 4
    
5.
Rossi GM, Rocco R, Accorsi Buttini E, Marvisi C, Vaglio A. Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. Intern Emerg Med 2017;12:287-99.  Back to cited text no. 5
    
6.
Vaglio A, Maritati F. Idiopathic retroperitoneal fibrosis. J Am Soc Nephrol 2016;27:1880-9.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]



 

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