Treatment of idiopathic retroperitoneal fibrosis

Abstract

Introduction: Idiopathic retroperitoneal fibrosis (RPF) is a rare condition hallmarked by a periaortic fibro-inflammatory tissue that often causes ureteral obstruction. Its clinical manifestations include abdominal or back pain, systemic symptoms such as fatigue, weight loss and fever and, less frequently, constipation, testicular pain, claudicatio and edema of the lower limbs. The primary goal of treatment is the relief of ureteral obstruction and the regression of the fibro-inflammatory process. The treatment is still empiric because, to date, there is only one randomized controlled trial. Patients often need long-term therapy because this disease may have a chronic-relapsing course.

Areas covered: This review summarized current strategies for the treatment of idiopathic RPF. An overview of the different therapeutic protocols is provided and the most relevant published references have been considered.

Expert opinion: Idiopathic RPF is potentially curable with the available treatments. It usually responds to glucocorticoids but patients with suboptimal responses to these drugs or multiple relapses should be treated with alternative approaches, including tamoxifen or immunosuppressive drugs.

Keywords::

• glucocorticoids
• immunosuppressive drugs
• obstructive uropathy
• relapsing disease
• retroperitoneal fibrosis
• tamoxifen

Notes

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