During the last 10 years drug treatment of idiopathic retroperitoneal
fibrosis (IRPF) has gained increasing importance as surgical measures
alone are fraught with recurrence rates of about 50%. Prednisone in an
initial dosage of 40-50 mg/day and 5-10 mg/day thereafter is the undi
sputed drug of choice. Additional administration of azathioprin (50 mg
/day) and aldosterone (300 mg/day) has been questioned since its effic
acy has not been proven. Recent reports of tamoxifen as a successful d
rug (20 mg/day) are as yet too rare to be evaluated. A close follow-up
is warranted to determine when treatment should be terminated either
due to disease regression or cortison side effects.