The intriguing co-existence of a chronic periaortitis, a pericarditis and a pan...

• Author(s): Rebelo, M ; Lima, J ; Ramos, L ; Vieira, JD ; Nascimento-Costa, JM
• Date: 2011
• Persistent ID: http://hdl.handle.net/10400.4/1163
• Origin: Repositório do Centro Hospitalar e Universitário de Coimbra
• Subject(s): Fibrose Retroperitoneal; Pericardite; Pancreatite
• Document Type: Article
• Language: English

Description

Chronic periaortitis (CP) refers to a spectrum of diseases whose common denominator is a fibro-inflammatory tissue developing in the periaortic space and frequently encasing surrounding structures like the kidney and ureters. There is no unified concept regarding the primary aetiology of CP, but recent studies have demonstrated that CP may present features of auto-immune diseases. CP involves three main entities, namely idiopathic retroperitoneal fibrosis (IRF), inflammatory aneurysms of the abdominal aorta (IAAAs) and perianeurysmal retroperitoneal fibrosis (PRF). These entities are usually diagnosed using computed tomography or magnetic resonance imaging, which typically show a retroperitoneal mass surrounding the aorta and that extends laterally without displacing it. Positron emission tomography is useful for the full assessment of the extent of the disease and its metabolic activity. The inflammatory and chronic relapsing nature of these diseases compels the use of medical therapy, which is based on high-dose steroids with a tapering scheme combined with immunosuppressive agents in refractory or relapsing disease. The authors report the clinical and radiological characteristics of a nonaneurysmatic form of chronic periaortitis in a woman presented with pericarditis, pericardial effusion and a pancreatitis. They also describe the investigation and management of this unusual condition