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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
Main Authors: Burkhardt Soares, S, Kukuk, S, Brandt, A S, Fehr, A, Roth, S
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Language:eng ; ger
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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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ispartof Urologe. Ausgabe A, 2008-04, Vol.47 (4), p.489
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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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