A case of localized abdominal aortic dissection secondary to retroperitoneal fibrosis cured only with glucocorticoids and immunosuppressive agents

[摘要] Aortic dissection is a life-threatening disease resultingfrom an intimal tear in most cases or a rupture of the vasavasorum and an intramural hematoma that may form anaortic dissection. Although the two main mechanisms areextracellular matrix degradation and inflammation, theprecise trigger of aortic dissection remains unknown (1).Contributing factors are diverse, and arterial hypertensionand known connective tissue diseases are the most commonrisk factors (2,3). Additionally, retroperitoneal fibrosis (RPF)is a rare condition whose central feature is the presence ofa fibro-inflammatory tissue arising from the outer layers ofthe abdominal aorta and extending into the surroundingretroperitoneum (4). RPF has been deemed a manifestationof a systemic immune-mediated or inflammatoryprocess rather than an exaggerated local reaction toatherosclerosis (5). The typical macroscopic appearance ofRPF is a retroperitoneal plaque of varying thickness thatsurrounds the abdominal aorta, the iliac vessels, and, inmost instances, the inferior vena cava and the ureters. Theaortic wall also undergoes atherosclerotic degeneration ofthe intima, medial thinning, and pronounced adventitialinflammation.

[学科分类] 外科医学