Narrative ReviewIdiopathic Retroperitoneal Fibrosis: A Review of the Pathogenesis and Approaches to Treatment
Section snippets
Pathogenesis of Idiopathic Disease
Review of the literature9, 11, 29, 44 suggests that in patients with retroperitoneal fibrosis, less than 25% will have an identifiable primary disease process, such as aortic aneurysm, malignancy, previous urinary infection, obstruction, immunologic, or inflammatory syndrome, or use of a known offending pharmacological agent. In the remainder of cases, the cause is unknown, consigning these cases to the idiopathic category, or IRPF.
Histological examination of IRPF specimens generally shows
General Approach to Treatment
The initial approach to IRPF often requires surgical, urological, or medical intervention to address anatomic complications. This may supersede the initiation of medical therapy directed at the underlying inflammatory and fibrotic process. For example, ureteral obstruction usually responds to stent placement, but sometimes requires ureterolysis5, 6, 8, 9, 10, 29, 57, 58 or even occasional nephrectomy; bowel obstruction requires evaluation, observation, and even diversion before
Recommendations and Conclusions
Previous teaching has characterized IRPF as rare, often difficult to diagnose, complicated to treat, and boding a guarded prognosis. However, review of the growing body of evidence, albeit uncontrolled, strongly suggests several important principles concerning IRPF: (1) the condition may be more common than we thought; (2) disease activity appears to be an idiopathic inflammatory and fibrotic response of autoimmune type with as yet unidentified inciting pathogenesis, and although some
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Originally published online as doi: 10.1053/j.ajkd.2009.04.019 on June 11, 2009.