Long-Term Renal and Patient Outcome in Idiopathic Retroperitoneal Fibrosis Treated With Prednisone

doi:10.1053/j.ajkd.2007.02.268

American Journal of Kidney Diseases

Volume 49, Issue 5, May 2007, Pages 615-625

https://doi.org/10.1053/j.ajkd.2007.02.268 Get rights and content

Background

Primary medical treatment of idiopathic retroperitoneal fibrosis (RPF) increasingly is accepted. However, the optimum treatment strategy is still unclear.

Study Design

Case series.

Setting & Participants

Single tertiary care referral center. 24 patients with idiopathic RPF treated with prednisone for 1 year, if needed, with (urgent) renal drainage from June 1991 through October 2006.

Outcome & Measurements

Clinical improvement, laboratory parameters, repeated computed tomographic (CT) scanning. Treatment was considered successful if the following criteria were met at the end of the 1-year treatment period: significant subjective clinical improvement, (near-)normalization of acute-phase reactants, improvement in renal function with disappearance of ureteral obstruction, and CT-documented mass regression. Recurrence is defined as need for retreatment because of return of signs and symptoms after the 1-year treatment period in patients with initial treatment success.

Results

22 patients reported significant to complete resolution of symptoms after median treatment duration of 2.0 weeks (0.7 to 3.0). Follow-up showed decreases in erythrocyte sedimentation rate, C-reactive protein level (both P < 0.0001), and serum creatinine level (P = 0.0230) at 6 weeks, which persisted during the treatment period. Repeated CT scanning showed mass regression in 19 patients during the treatment period. Six patients were considered treatment failures, and there were 23 recurrences 10 months (7 to 14) after prednisone withdrawal in 13 of 18 patients with initial treatment success. At the end of follow-up (median, 55 months), 7 patients had impaired renal function; 1 patient reached end-stage renal disease. The mortality rate was 8%.

Limitations

There was no comparison with other treatments.

Conclusion

One-year treatment with prednisone is associated with a high rate of initial success, but a high recurrence rate. Despite frequent disease relapse, long-term renal and patient outcome was good.

Section snippets

Patients

Twenty-four patients with idiopathic RPF treated with prednisone monotherapy for first presentation of disease from June 1991 through October 2006 are the subject of this retrospective study. All patients were followed up by our nephrology team at a single tertiary-care referral center. Fourteen study patients were referred to us from other departments (general internal medicine, gastroenterology, and vascular surgery).

All study patients had a clinical and radiological diagnosis of RPF in the

Results

Demographic and clinical characteristics of the 24 patients are listed in Table 1. Median patient age was 56 years (IQR, 52 to 64 years), with a male predominance (83%). Median duration of symptoms before diagnosis was 4 months (IQR, 2.5 to 6.0 months). Median follow-up was 55 months (IQR, 29 to 79 months). Posttreatment follow-up amounted to 45 months (IQR, 23 to 77 months). During careful follow-up, no other pathological state was found that could have accounted for the findings at

Discussion

The present study shows that conservative treatment with prednisone for 1 year and urgent renal drainage, if necessary, leads to satisfactory initial results in up to 75% of patients with idiopathic RPF. Most patients reported amelioration of symptoms and had decreases in acute-phase reactant levels and improvement in renal function within a few weeks or, in some cases, a few days. CT-documented mass regression usually was observed within 6 months of therapy, and in some cases, within 3 months.

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: Originally published online as doi:10.1053/j.ajkd.2007.02.268 on April 9, 2007.

: Support: None. Potential conflicts of interest: None.

View full text

Original InvestigationPathogenesis and Treatment of Kidney DiseaseLong-Term Renal and Patient Outcome in Idiopathic Retroperitoneal Fibrosis Treated With Prednisone

Background

Study Design

Setting & Participants

Outcome & Measurements

Results

Limitations

Conclusion

Section snippets

Patients

Results

Discussion

Lancet

J Urol

Presse Med

Am J Med

Retroperitoneal fibrosisAn update

Dig Dis

Retroperitoneal fibrosisA true connective tissue disease

Rheum Dis Clin North Am

Retroperitoneal fibrosis

Neth J Med

Auto-allergic periaortitis (idiopathic retroperitoneal fibrosis)

BJU Int

What is steroid-responsiveness in retroperitoneal fibrosis?

J Am Soc Nephrol

Clinical parameters and therapeutic outcome in patients with idiopathic retroperitoneal fibrosis

Arthritis Rheum

Idiopathic retroperitoneal fibrosis: The case for non-surgical treatment

Br J Urol

Original Investigation
Pathogenesis and Treatment of Kidney Disease
Long-Term Renal and Patient Outcome in Idiopathic Retroperitoneal Fibrosis Treated With Prednisone