Takayasus's Aortitis Induced Ascending Aorta Dissection

Takayasu aortitis is a well known yet rare form of large vessel vasculitis. Also known as pulseless disease, occlusive thromboaortopathy, and Martorell syndrome, is a chronic inflammatory aortitis. Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation.

A 64-year-old woman was referred to emergency for lack of pulse in the upper and lower limbs and changes in heart rate. AngioRMN revealed dissection of the ascending aorta while in PET, intense uptake of FDG-F18 involving ascending, crossa, descending thoracic and abdominal segments of the aorta, was evident urgent surgical correction occurred. An aorta ring segment with 2.5cm length, showed whitish and smooth intimate, with linear transversal laceration, with regular borders. Dissection 1cm long of the medial tunica was occupied by a clot in continuity with a thrombus occupying the neoformed lumen.

Microscopy examination confirmed hyalinization of the tunica media with impregnation of fibrin / thrombus with blood cell elements. Endothelial inflammatory characteristics together with vasa vasorum and vascular trajectory of the periphery of the tunica media with inflammatory cells involvement allowed the diagnosis of Takayasu aortitis.

Takayasu aortitis is rare in the presented age group, with early non-specific symptoms. The diagnosis of aortic dissection was crucial, constituting a medical emergency. Heather L-Gomik (2008) supports the hyaline structural alteration of the tunica media. The disease has been recognized for more than 100 years, and patients with Takayasu aortitis remain relatively poor and treatment is suboptimal. Key areas for improvement include the need for increase disease awareness and earlier diagnosis, and improved means for monitoring disease activity. The demonstration of diferential expression of Toll-like receptors in arteries, is particularly intriguing and worthy of further investigation.