[New (and old) aspects of retroperitoneal fibrosis]. / Neue (und alte) Aspekte der retroperitonealen Fibrose.

Retroperitoneal fibrosis (RPF) is an uncommon chronic inflammatory disease of the rear abdomen and it is commonly associated with the complication of uni- or bilateral hydronephrosis. Despite the rarity of this disease, multiple publications concerning etiology, diagnosis, therapy monitoring and both medical and surgical therapy have been published in recent years. Recent research has focused on asbestos exposure as a possible risk factor, the meaning of IgG4-associated RPF, measuring disease activity using different radiological and nuclear medicine procedures, and new approaches to medical therapy. Goals of treatment were characterized as the correct diagnosis, preservation of renal function and freedom from stents, steroids and pain. On the basis of these goals, the most important insights and developments of the last 5 years regarding RPF are presented.

[Diagnosis and treatment of retroperitoneal fibrosis]. / Diagnostik und Therapie der retroperitonealen Fibrose.

Retroperitoneal fibrosis (RPF) is a rare chronic inflammatory disease of the posterior abdominal cavity. Hydronephrosis due to extrinsic compression of the ureter is the most frequent complication of the disease, which is why patients are often treated by urologists.On the basis of checklists the most important and fundamental facts of diagnosis (symptoms, hydronephrosis, laboratory parameters, diagnostic radiology, histology, renal function and measuring disease activity) and therapy (goals of therapy, medical and surgical therapy) are presented.

[Therapy of retroperitoneal fibrosis: functional therapeutic outcome]. / Therapie der retroperitonealen Fibrose : Funktionelle Behandlungsergebnisse.

BACKGROUND: The most frequent complication of retroperitoneal fibrosis (RPF) is an extrinsic compression of the ureter resulting in hydronephrosis. Because of the rareness of this uncommon chronic inflammatory disease, therapeutic results concerning the treatment of the obstructive uropathy are often known only for small study groups. MATERIAL AND METHODS: The Else Kröner-Fresenius registry is a nationwide register of patients suffering from RPF in which all disease-related data are prospectively recorded. We performed a retrospective analysis of treatment outcome of different therapy strategies for all patients who underwent standardized treatment in our department since 2007. RESULTS: From April 2007 to January 2014 a total of 97 patients with RPF were treated in our department. The mean age was 54.9 years and the male to female ratio was 3.2:1 (74 males and 23 females). Assessment of 85 out of 97 cases revealed 78 patients and 118 renal units with hydronephrosis. Of these patients 46 received prednisolone, 35 patients tamoxifen and 4 patients a combination therapy. Successful removal of double J stents was possible in 49 out of the 78 cases (62.8%) and in 72 out of 118 renal units (61.0%). Of the patients 26.9% received final surgical treatment of ureter compression and 7.7% of patients were permanently treated with a double J stents. Patients treated with prednisolone were significantly younger (p=0.040) and decided for surgical therapy significantly earlier (p=0.041). Otherwise there were no differences in functional outcome of patients treated with either prednisolone or tamoxifen. CONCLUSION: Removal of double J stents was possible in 63% of patients regardless of the medicinal approach to therapy, which supports the strategy of using medicinal treatment before the application of surgical solutions. Nevertheless, surgical interventions were necessary in more than 25% of cases.

[Retroperitoneal fibrosis: development of a biomarker profile for diagnosis and therapy monitoring]. / Retroperitoneale Fibrose : Entwicklung eines Biomarkerprofils zur Diagnostik und Verlaufskontrolle.

INTRODUCTION: Retroperitoneal fibrosis (RPF) is a rare chronic inflammatory disease which is characterized by fibrotic tissue in the retroperitoneal space. There have only been a few studies on serum markers of this disease. The main goal of the current investigation was to identify biological markers which are increased in the serum of patients suffering from RPF and which may correlate with the extent of fibrosis. MATERIAL AND METHODS: The serum of 42 patients with primary and yet untreated retroperitoneal fibrosis was examined for biomarkers known to be specific for fibrotic diseases and compared to a control group. In addition, patients were stratified according to the extent and volume of the retroperitoneal mass using cross-sectional imaging. To estimate the discriminatory power of the evaluated biomarkers, receiver operating characteristic (ROC) curves were created. RESULTS: Independent of the extent of fibrosis, calprotectin, fibrinogen, osteopontin, matrix metallopeptidase 9 (MMP-9), tenascin C and TIMP metallopeptidase inhibitor 1 (TIMP-1) were significantly increased (p<0.01) in patients suffering from RPF compared to the control group. Connective tissue growth factor (CTGF) was significantly elevated (p<0.01) in patients with high RPF burden only but monocyte chemoattractant protein 1 (MCP-1) and heart-type fatty acid binding protein (H-FABP) showed no increase in serum levels. The discriminatory power of these parameters was ranked by the ROC analysis which demonstrated an area under the curve (AUC) >0.87 for MMP-9, TIMP-1, osteopontin, tenascin C, asymmetric dimethylarginine (ADMA), fibrinogen and calprotectin and an AUC <0.64 for MMP-2, CTGF, H-FABP and MCP-1. CONCLUSION: Several biomarkers of fibrogenesis were significantly elevated in patients suffering from RPF as compared to a control group. These biomarker candidates will be further evaluated for their potential to allow a differentiation between other diseases or if they could be used for disease monitoring.

MR evaluation of retroperitoneal fibrosis.

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.

Associated findings and complications of retroperitoneal fibrosis in 204 patients: results of a urological registry.

PURPOSE: We studied the frequency of associated findings and complications of retroperitoneal fibrosis in a large clinical collective of urological patients. MATERIALS AND METHODS: All urology departments in Germany were invited to participate in a registry of patients with retroperitoneal fibrosis, for which a data sheet with more than 200 questions was developed. RESULTS: As of March 2010 a total of 204 patients were registered. The male-to-female ratio was 2.1:1 and the average age at onset was 55.6 years. In 123 cases (60.3%) the diagnosis was confirmed by histopathology. Coexisting autoimmune diseases were found in 9.8% of patients, consisting mainly of thyroid disorders. Coexisting fibrosis was detected in 3.4% of the patients and 73.9% of those queried were active smokers. Of 176 patients for whom data on therapy were available 123 received monotherapy and 41 received combination therapy for a mean of 12.1 months (12 received none), while 87 underwent a total of 103 operative procedures. Hydronephrosis was the most frequent complication in 95.6% of patients. Atrophic kidney from undetected hydronephrosis appeared in 46 patients (22.5%) and in 4 bilateral damage necessitated dialysis. Complications from vascular obstruction were observed in 27.5% of patients. Large bowel obstruction requiring colostomy occurred in 4 patients (2.0%). CONCLUSIONS: Patients with retroperitoneal fibrosis often first present to urology departments upon referral for hydronephrosis. In this series at least 1 kidney appeared to be irreversibly damaged in more than 20% of patients. Urologists should be mindful of the role of smoking role as a risk factor, complications arising from vascular and large bowel obstructions, and the possible association of retroperitoneal fibrosis with autoimmune disease.

[Retroperitoneal fibrosis]. / Retroperitoneale Fibrose.

Retroperitoneal Fibrosis (RPF) is a rare disease with peak incidence in the fifth to seventh decades of life. Its etiology remains to a great extent unclear; however, many characteristics of the disease suggest an autoimmune origin. Distinction is made between primary/idiopathic and secondary RPF. Symptoms may be general/nonspecific or localized (due to replacement or compression of organs). In addition to patient history, laboratory analysis and radiologic examinations, diagnosis often requires biopsy taken from the area affected. After restoring the function of involved (hollow) organs, medical therapy with prednisone, immunosuppressive drugs or tamoxifen is aimed at converting active disease to stable disease. However, some patients require ureteral surgery due to unsuccessful treatment. Life-long follow-up of patients is necessary since RPF can show a chronically relapsing course.