A Case of Idiopathic Retroperitoneal Fibrosis with Acute Renal Failure / 대한신장학회잡지

ABSTRACT

Retroperitoneal fibrosis is proliferation of fibrous tissue with inflammatory process in retroperitoneal cavity. It is relatively rare disease that has been reported less than 20 cases in Korea until now. Idiopathic type is more frequent but secondary type is increasing nowadays. Secondary causes include drugs, infections, and leakage of blood or urine, malignancies, connective tissue diseases, etc. Recent studies suggest the relationship between retroperitoneal fibrosis and autoimmunity to own vascular or lipoid tissue. It can cause compression and obstruction of ureter, abdominal aorta, hypertension and finally collapse of renal function. Surgical procedure and immunosuppressive therapy consist of mainstay of management. Corticosteroid therapy may reduce inflammation and reverse fibrosis. Retroperitoneal fibrosis is thought to have some reVersible components in early stage. Corticosteroid may be used as initial therapy but more studies should be performed. We report a case of idiopathic retroperitoneal fibrosis with acute renal failure improved with ureter stent insertion and steroid therapy.