A case of mediastinal fibrosis in a patient with idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis is a rare disease characterized by proliferation of fibrous tissue with inflammation in the retroperitoneal cavity, which is often associated with fibrosis of other organ systems. Multifocal fibrosclerosis is a term used to describe a combination of multiple organ system fibrosis, such as mediastinal fibrosis, retroperitoneal fibrosis, orbital pseudotumor, sclerosing cholangitis, and Reidel’s thyroiditis. Although there are several reports of retroperitoneal fibrosis associated with a combination of fibrosis in variable organ systems, reports of combined retroperitoneal fibrosis and mediastinal fibrosis are rare. We report a case of mediastinal fibrosis in a patient with idiopathic retroperitoneal fibrosis. (Korean J Med 77:S1258-S1262, 2009)