Combined idiopathic retroperitoneal and mediastinal fibrosis with pericardial involvement.

ABSTRACT

We report the case of a 54-year-old woman with renal failure related to bilateral hydronephrosis. Clinical and radiologic findings were consistent with retroperitoneal fibrosis associated with pleuro-pericardial involvement. These features matched criteria for combined idiopathic retroperitoneal fibrosis and idiopathic mediastinal fibrosis. There are few reported cases of pericardial involvement in this combined disorder. In our observation, nuclear-magnetic resonance was the radiologic procedure of choice for aortic exploration. The responsibility of therapeutic agents (beta-adrenoreceptor-blocking drugs and ergotamine) and therapeutic options for the sclerosing disease are also discussed. Bilateral ureteral stents and corticosteroids produced favorable outcome without recurrence one year after treatment was stopped.