Treatment of idiopathic retroperitoneal fibrosis using cyclosporin

Abstract

The main goal of traditional treatment of idiopathic retroperitoneal fibrosis is limitation of morbidity, and surgery of already formed fibrous masses has been the main therapeutic approach. More recently, the knowledge that the disorder may be the result of an allergic reaction to atherosclerotic lipids has prompted the use of corticosteroids and cytotoxic drugs, which proved efficacious, but also toxic. On the basis of data indicating a T cell pathogenesis of idiopathic retroperitoneal fibrosis, cyclosporin, a non-cytotoxic pretranscriptional inhibitor of proinflammatory cytokines, was used to treat the case reported here. A 65 year old man with aggressive retroperitoneal fibrosis and obstructive renal failure initially received steroids, which eventually lost their efficacy and led to vertebral collapse. He responded to 5 mg/kg/day cyclosporin, with radiological reduction of tissue deposition, relief of urether compression, and reduction in acute phase reactants in the blood. Chronic disease remission required stable drug concentrations. In conclusion, progress in research into the T cell pathogenesis of idiopathic retroperitoneal fibrosis may justify attempts with drugs such as cyclosporin to block the disease at its origin rather than treating the morbidity.