The salient pathological findings in 40 patients with idiopathic retroperitoneal fibrosis are summarized. The findings tend to confirm previous suggestions that this chronic inflammatory disease is potentially widespread, predominantly peri-aortic, associated with systemic disturbances, and of some fundamental interest. The name `systemic idiopathic fibrosis' is suggested for the disease complex. The study suggests that damage to the wall of the aorta and its large branches might be the cause.
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