[Retroperitoneal fibrosis and generalized scleroderma]

A Raynaud's phenomenon was observed in a 72 year-old man affected by progressive systemic sclerosis. Few months later, the patient was readmitted to the Hospital because of acute renal failure, and a retroperitoneal fibrosis was first suspected on the basis of typical urinary tract alterations, and subsequently confirmed at the post-mortem examination. Such association does not appear fortuitous on histological data, but it has been rarely described. HLA B27 antigen is not always present. Symptomatic therapy includes association between prednisone and percutaneous nephrostomy.