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Retroperitoneal fibrosis
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. S...
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Published in: | Urologe. Ausgabe A 2008-04, Vol.47 (4), p.489 |
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container_title | Urologe. Ausgabe A |
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creator | Burkhardt Soares, S Kukuk, S Brandt, A S Fehr, A Roth, S |
description | 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. |
doi_str_mv | 10.1007/s00120-008-1705-6 |
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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.</description><identifier>ISSN: 0340-2592</identifier><identifier>DOI: 10.1007/s00120-008-1705-6</identifier><identifier>PMID: 18365166</identifier><language>eng ; ger</language><publisher>Germany</publisher><subject>Aged ; Aortic Aneurysm, Abdominal - diagnosis ; Aortic Aneurysm, Abdominal - pathology ; Aortic Aneurysm, Abdominal - therapy ; Autoimmune Diseases - diagnosis ; Autoimmune Diseases - pathology ; Autoimmune Diseases - therapy ; Biopsy ; Child ; Comorbidity ; Diagnosis, Differential ; Female ; Humans ; Immunosuppressive Agents - therapeutic use ; Kidney Function Tests ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Prednisone - therapeutic use ; Recurrence ; Retroperitoneal Fibrosis - diagnosis ; Retroperitoneal Fibrosis - pathology ; Retroperitoneal Fibrosis - therapy ; Tomography, X-Ray Computed ; Ureter - pathology ; Ureter - surgery ; Ureteral Obstruction - diagnosis ; Ureteral Obstruction - pathology ; Ureteral Obstruction - therapy ; Urography</subject><ispartof>Urologe. 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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. 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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.</abstract><cop>Germany</cop><pmid>18365166</pmid><doi>10.1007/s00120-008-1705-6</doi></addata></record> |
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subjects | Aged Aortic Aneurysm, Abdominal - diagnosis Aortic Aneurysm, Abdominal - pathology Aortic Aneurysm, Abdominal - therapy Autoimmune Diseases - diagnosis Autoimmune Diseases - pathology Autoimmune Diseases - therapy Biopsy Child Comorbidity Diagnosis, Differential Female Humans Immunosuppressive Agents - therapeutic use Kidney Function Tests Magnetic Resonance Imaging Male Middle Aged Prednisone - therapeutic use Recurrence Retroperitoneal Fibrosis - diagnosis Retroperitoneal Fibrosis - pathology Retroperitoneal Fibrosis - therapy Tomography, X-Ray Computed Ureter - pathology Ureter - surgery Ureteral Obstruction - diagnosis Ureteral Obstruction - pathology Ureteral Obstruction - therapy Urography |
title | Retroperitoneal fibrosis |
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