Treatment of retroperitoneal fibrosis
- Augusto Vaglio, MD, PhD
Augusto Vaglio, MD, PhD
- University Hospital, Parma, Italy
- Alessandra Palmisano, MD, PhD
Alessandra Palmisano, MD, PhD
- Department of Clinical and Experimental Medicine
- University of Parma, Italy
Retroperitoneal fibrosis, also referred to as Ormond's disease, is an uncommon but treatable cause of obstructive uropathy. This disorder involves chronic inflammation and fibroblast proliferation, with excessive extracellular matrix deposition. Untreated patients may develop severe complications or progress to end-stage renal disease (ESRD) .
Retroperitoneal fibrosis is also sometimes associated with venous or arterial occlusion due to compression, or abdominal aortic aneurysms, if in the setting of chronic periaortitis. In addition to medical therapy for the retroperitoneal fibrosis, these situations may require endovascular or surgical management. The management of these issues requires consultation with the appropriate specialists. (See "Clinical manifestations and diagnosis of retroperitoneal fibrosis".)
The treatment of idiopathic retroperitoneal fibrosis is reviewed here. The clinical manifestations, pathogenesis, and diagnosis of this disorder are presented separately. (See "Clinical manifestations and diagnosis of retroperitoneal fibrosis".)
GENERAL APPROACH TO THERAPY
The goals of therapy are to relieve the obstruction caused by fibrosis, stop the progression of the fibrotic process, and prevent recurrence.
If the retroperitoneal fibrosis is due to a secondary cause (table 1), treatment is aimed at the underlying etiology. If the disease is idiopathic, we initiate immunosuppressive therapy.
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- GENERAL APPROACH TO THERAPY
- INTERVENTION IN PATIENTS WITH URINARY TRACT OBSTRUCTION
- Selection of an interventional procedure
- Monitoring the response to interventional therapy
- MEDICAL THERAPY
- Medical treatment of secondary disease
- Medical treatment of idiopathic disease
- - Initial therapy with glucocorticoids
- Glucocorticoid-resistant disease
- - Initial therapy with tamoxifen in patients who cannot take glucocorticoids
- - Duration of therapy
- - Alternative initial therapy with combined immunosuppression
- - Investigative therapies
- Monitoring the response to medical therapy
- Recurrence after discontinuation of therapy
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS