An inflammatory disorder of the aortic arch and its major branches, with localized stenosis or occlusion
An auto-immune basis has been proposed. It is most common in young women, similar to other auto-immune disorders. Presence of giant cells in the lesion makes the diagnosis difficult but the clinical presentation helps to differentiate.
Pathogenesis : Also known as PULSELESS DISEASE.
There is irregular thickening of the aortic arch or branch vessel wall with intimal wrinkling.
When the aortic arch is involved, the orifices of the major arteries to the upper portion of the body may be markedly narrowed or even obliterated by intimal thickening – which leads to weakness of pulse in upper extremity – pulseless disease.