Outcome of patients with idiopathic retroperitoneal fibrosis treated with tamoxifen or corticosteroid monotherapy
Bilt, F.E. van der, Meijden, W.A.G. van der, Hendriks, T.R., Bommel, E.F.H. van
Voorzitter(s): dr. P.M. Netten, ‘s-Hertogenbosch & dr. L.J.M. Reichert, Arnhem
Locatie(s): Auditorium 1
Categorie(ën):
Objective: Idiopathic retroperitoneal fibrosis (iRPF) is a rare chronic inflammatory disorder of unknown etiology. Although corticosteroids are used most often as primary therapy, tamoxifen might be a suitable alternative, particularly in the presence of contra-indications for long-term use of corticosteroids. No comparative data of these two drugs as monotherapy are available. We compared outcome of iRPF patients treated with corticosteroid or tamoxifen monotherapy for first presentation.
Methods: Of all patients with iRPF disease who were referred to our tertiary care referral centre from February 1999 through December 2011, 118 patients were eligible for this retrospective study. Treatment success was defined as the composite of: (1) amelioration of symptoms; (2) CT-documented mass regression; and, if applicable (3) definitive removal of ureteral stent or nephrostomy tube. Recurrence was defined as recurrence of signs and symptoms and/or CT-documented mass increase after initial treatment success with primary treatment.
Results: Presenting signs and symptoms did not differ between patients treated with corticosteroids (CS) (n=50) or tamoxifen (TAM) (n=68). In patients treated with corticosteroids, median (IQR) ESR (CS, 64 [40-95] mm/h vs. TAM, 42 [15-79] mm/h ; P < 0.01) and CRP levels (CS, 44 [16-99] mg/L vs. TAM, 10 [5-34] mg/L; P<0.001) were higher at presentation. Serum creatinine level did not differ between groups (CS, 128 [90-205] µmol/L) vs. TAM, 111 [92-141] µmol/L; P=0.19). Time to resolution of symptoms after treatment initiation was shorter in corticosteroid-treated patients (CS, 2.0 [0.8-3.8] wk vs TAM 4.0 [2.0-6.0] wk; P<0.01). Mass regression at first follow-up CT scan tended to be observed more frequently in patients treated with corticosteroids compared to patients treated with tamoxifen (CS, 84.0% vs. TAM, 68.3%; P = 0.054). Median time-interval from treatment initiation to first follow-up CT scan did not differ between groups (CS, 5 [2-7] mo vs. TAM, 4 [4-5] mo; P=0.34). Treatment success did not differ significantly between patient groups (CS, 72.7% vs. TAM, 58.3%; P=0.15). In patients with initial treatment success with primary treatment, recurrence rate was lower in patients treated with tamoxifen (CS, 62.5% vs. TAM, 21.4%; P<0.01)
Conclusion: More rapid resolution of symptoms and more frequent mass regression at first follow-up CT scan was observed in patients treated with corticosteroids. Percentage of treatment success was non-significantly higher in corticosteroid-treated patients. Conversely, in patients who had initial treatment success with primary treatment, recurrence rate was lower in tamoxifen-treated patients.