Peri-aortic fibrosis: progression and regression

The natural history and aetiology of peri-aortic fibrosis is unknown. In one of our patients in whom steroid treatment had successfully controlled the progression of the disease' cessation of treatment was associated with recurrence of the fibrosis, progressive ureteric obstruction and dilatation of the aorta. In another patient, graft replacement of the aorta was associated with regression of the fibrosis but in a third patient replacement of the aorta did not prevent progression to complete ureteric obstruction. It would seem that it is the presence of the fibrosis that engenders the aortic dilatation not the presence of a patent aorta, dilated or not.