Retroperitoneal fibrosis revisited.

Area of Science:

• Pathology
• Vascular Biology
• Autoimmunity

Background:

• Idiopathic retroperitoneal fibrosis (IRF) shares histopathologic features with mediastinal fibrosis, perianeurysmal fibrosis, and inflammatory aortic aneurysm.
• These conditions are proposed to be unified under the term "chronic periaortitis," with coronary involvement termed "chronic periarteritis."

Purpose of the Study:

• To consolidate the understanding of idiopathic retroperitoneal fibrosis and its related conditions.
• To explore the potential autoimmune etiology of chronic periaortitis.

Main Methods:

• Histopathologic analysis of patient cases.
• Review of existing literature and proposed etiological theories.

Main Results:

• Histopathologic evidence indicates that IRF, idiopathic mediastinal fibrosis, perianeurysmal retroperitoneal fibrosis, and inflammatory aneurysm of the aorta are essentially identical.
• The term "chronic periaortitis" encompasses these variants, with "chronic periarteritis" describing associated coronary artery involvement.
• A proposed autoimmune reaction to atherosclerotic plaque components is supported by some recent evidence, though not definitively proven.

Conclusions:

• Chronic periaortitis is a unifying term for a spectrum of fibrotic diseases.
• Surgical ureterolysis is the preferred treatment for uncomplicated cases.
• The disease demonstrates significant sensitivity to corticosteroid therapy.