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Takayasu arteritis, which is also known as aortic arch syndrome, non-specific aortoarteritis and pulseless disease, is a rare chronic inflammatory progressive large vessel vasculitis of likely autoimmune aetiology causing narrowing, occlusion and aneurysms of systemic and pulmonary arteries, especially the aorta and its branches, afflicting women of childbearing age. It is an uncommon disease with an estimated annual incidence rate of 1.2–2.6 per million. Hypertension is the the most common mode of presentation. Based on site and extent, it is divided into five types as type I (branches of aortic arch), type II (aortic arch, its branches and descending thoracic aorta), type III (descending thoracic aorta and abdominal aorta), type IV (abdominal aorta only) and type V (aortic arch, descending thoracic aorta and abdominal aorta). Therapeutic modalities include medical (antihypertensive, steroids, cyclophosphamide, etc) as well as surgical interventions.