Objective: To evaluate the therapeutic effect of tamoxifen monotherapy in patients with retroperitoneal fibrosis (RPF). Patients and Methods: From 2007 on, 31 patients with idiopathic RPF were treated with tamoxifen monotherapy. Follow-up investigations included magnetic resonance imaging, laboratory measurements, registration of side effects and changes or removal of ureteral stents. Data were stored in the Else Kröner-Fresenius Registry of Retroperitoneal Fibrosis. Results: 25 men and 6 women with a mean age of 56.6 years were treated with tamoxifen monotherapy. Mean duration of treatment was 13.3 months, mean follow-up 26.8 months. A total of 44 renal units were affected by hydronephrosis and covered by DJ stents. Radiological regression of fibrosis was detected in 22 cases (71.0%); removal of ureteral stents was possible in 27/44 renal units (61.4%) and 17/29 patients (58.6%), respectively. Most patients showed only mild or no side effects of therapy. In 7 cases (22.3%) tamoxifen therapy had to be abandoned because of severe side effects, progression of fibrosis or persistent intolerance. Conclusions: Tamoxifen is an alternative in the medical treatment of RPF, especially if patients want to avoid glucocorticoids. The potential of regression of fibrosis seems to be slightly inferior and the relapse rate is higher compared to steroids, but the rate of successful DJ removals is comparable.

To date there is no validated evidence for standardized treatment of patients with Takotsubo syndrome (TTS). Medication therapy after final TTS diagnosis remains unclear. Previous data on patient outcome is ambivalent. Aim of this study was to evaluate medication therapy in TTS and to analyze patient outcome. Within an observational retrospective cohort study we analyzed our medical records and included 72 patients with TTS that underwent cardiovascular magnetic resonance imaging (CMR) after a median of 2 days interquartile range (IQR 1–3.5). We investigated medication therapy at discharge. Medication implementation and major adverse clinical events (MACE) were prospectively evaluated after a median follow-up of 24 months (IQR 6–43). Left ventricular function, myocardial oedema and late gadolinium enhancement were analyzed in a CMR follow-up if available. Antithrombotic therapy was recommended in 69 (96%) patients including different combinations. Antiplatelet monotherapy was prescribed in 28 (39%) patients. Dual antiplatelet therapy was recommended in 29 (40%) patients. Length of therapy duration varied from one to twelve months. Only in one case oral anticoagulation was prescribed due to apical ballooning with a left ventricular ejection fraction <30%. In all other cases oral anticoagulation was recommended due to other indications. ß-adrenoceptor antagonists and ACE inhibitors were recommended in 63 (88%), mineralocorticoid receptor antagonists were prescribed in 31 (43%) patients. After a median of 2 months (IQR 1.3–2.9) left ventricular function significantly recovered (49.1% ± 10.1 vs. 64.1% ± 5.7, P < 0.001) and myocardial oedema significantly decreased (13.5 ± 11.3 vs. 0.6% ± 2.4, P = <0.001) in the CMR follow-up. The 30-day mortality was 1%. MACE rate after 24 months was 12%. Although therapy guidelines for TTS currently do not exist, we found that the majority of patients were treated with antithrombotic and heart failure therapy for up to twelve months. Left ventricular function and myocardial oedema recovered rapidly within the first two months. Outcome analysis showed a low bleeding rate and a high short-term survival. Therefore, TTS patients might benefit from antithrombotic and heart failure therapy at least for the first two months.

Ziel: Die retroperitoneale Fibrose (RPF) ist eine seltene fibrosierende Erkrankung. Das Verhältnis von Kontrastmittelaufnahme zwischen RPF und dem M. psoas wird als Methode zur Aktivitätsbeurteilung und Verlaufskontrolle empfohlen, bislang gibt es allerdings keine standardisierten Parameter zur Beurteilung der Krankheitsaktivität. Material und Methoden: 29 Patienten mit unbehandelter, idiopathischer RPF wurden im MRT vor dem Beginn der medikamentösen Therapie und nach 3 Monaten untersucht. Die MR-Analysen umfassten Quotienten des T 1-Signals nach Kontrastmittelgabe und des T 2-Signals, jeweils zwischen RPF und M. psoas. Zusätzlich wurde die dynamische KM-Aufnahme der Fibrose untersucht. Die Daten wurden mit der Ausdehnung der RPF nach 3 Monaten verglichen. Ergebnisse: In der Verlaufsuntersuchung wurde eine deutliche Regression in 37,9 % (n = 11), eine leichter Rückgang in 48,3 % (n = 14) und eine unveränderte Ausdehnung der RPF in 13,8 % (n = 4) diagnostiziert. Patienten mit deutlich verminderter Ausdehnung zeigten in der Erstuntersuchung die höchsten Mittelwerte für die untersuchten MR-Quotienten. Die niedrigsten Werte für die entsprechenden Parameter wurden in der Gruppe mit unveränderter Ausdehnung der RPF beobachtet. Nur die dynamischen Enhancement-Quotienten zeigten statistisch signifikante Unterschiede (p = 0,011) zwischen den verschiedenen Gruppen. Schlussfolgerung: In Patienten mit erhöhten dynamischen Enhancement-Quotienten innerhalb der RPF wurde nach medikamentöser Therapie eine deutlich rückläufige Ausdehnung beobachtet. Purpose: Retroperitoneal fibrosis (RPF) is a rare fibrosing process. The relation of contrast enhancement within the fibrosis and the psoas muscle was suggested for the evaluation of disease activity and response to treatment, but no standardized method for the quantification of the disease activity is currently available. We introduce an MR parameter for the evaluation of RPF. Materials and Methods: 29 patients with untreated idiopathic RPF were examined before medical therapy and after three months of treatment. MR analyses included quotients of T 1 and T 2-signal intensities between the RPF and the psoas muscle. Furthermore, we examined the dynamic contrast enhancement of the fibrous tissue. The respective data were compared with the extent of the RPF after 3 months. Results: In the follow-up examinations we observed a significant regression in 37.9 % (n = 11) of cases, a mild regression in 48.3 % (n = 14) and a stable extent in 13.8 % (n = 4). Patients with a significantly reduced extent showed the highest mean values for the applied MR quotients in the initial examination. The lowest mean values for the respective parameters were found for the group with a stable extent. Only dynamic enhancement quotients showed statistically significant differences (p = 0.011) between the groups of response. Conclusion: We observed a significant reduction in the fibrous tissue after pharmacological treatment in patients with an elevated dynamic enhancement quotient in the initial examination.

Diagnostic and Interventional Radiology