Diseases Of The Aorta: Nonspecific Aortoarteritis
An idiopathic inflammatory process involving the aorta and its major branches may occur as a primary abnormality as in Takayasu’s arteritis or it may occur in association with conditions such as scleroderma, Hodgkin’s disease or rheumatoid arthritis. It may produce stenosis of aorta and its major branches or dilatation of aortic root with aortic regurgitation or aneurysm of thoracic or abdominal aorta.
Also known as Pulseless disease, young female arteritis or Harbitz-Raedu syndrome, is named after the Japanese ophthalmologist who first described changes in the central vessels of the retina. Women are affected 8 times more than men. More than half the patients are under 20 years of age. The disease is prevalent in India, Japan, south east Asia, south and east Africa, South America and Mexico.
Aorta In The Human Body
Pathology And Clinical Presentation
The histological findings include panarteritis, fibrous scarring, intimal proliferation, thrombosis and aneurysm resembling syphilitic aortitis, but there is no clinical or serologic evidence to suggest syphilitic aetiology even remotely. Current thinking favours an autoimmune process to be operative. Though tuberculosis was thought to play a role in its etiology, there is no evidence to confirm this view.
The presenting symptoms may be those associated with hypertension, viz, headache, giddiness, palpitation, congestive heart failure and pulmonary edema. Constitutional symptoms occur only during the early stage of the disease. Depending on the site of lesions, clinical manifestations may vary. When the arch and its vessels are involved, symptoms are due to ischemia to the brain and upper extremities. Pulse is absent in the upper limbs and there is hypertension in the lower extremities (reversed coarctation). Signs of aortic regurgitation may be present.
Middle Aortic Syndrome Representation
The Middle Aortic syndrome
This is the most prevalent type in India and it manifests by involvement of the descending thoracic or abdominal aorta with or without stenosis of the renal arteries or left subclavian. This picture may mimic congenital coarctation but presence of abdominal, subclavian or carotid bruit, difference in blood pressure between the upper extremities, claudication in the lower limbs and absence of collaterals around the scapula and rib notching suggests the diagnosis of aortoarteritis. Angina pectoris due to coronary arterial involvement, abdominal angina due to mesenteric involvement and pulmonary artery involvement occur uncommonly.
© 2014 Funom Theophilus Makama