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Takayasu arteritis (TAK), also known as idiopathic medial aortopathy or pulseless disease, is a granulomatous large vessel vasculitis that predominantly affects the aorta and its major branches. It may also affect the pulmonary arteries. The exact cause is not well known but the pathology is thought to be similar to giant cell arteritis.
There is a strong female predominance (F: M ~ 9:1), an increased prevalence in Asian populations, and it tends to affect younger patients (<50 years of age). The typical age of onset is at around 15-30 years of age.
Interestingly, it has been noted that there is a degree of geographic concurrence of cases of tuberculosis and Takayasu arteritis, suggesting a link between them. Perhaps TB triggers an immune-mediated reaction to the large vessels? This hypothesis remains contentious 12.
It induces clinically varied ischemic symptoms due to stenotic lesions or thrombus formation. The exact spectrum can be highly variable and dependent on the territory of vascular involvement.
There is segmental and patchy granulomatous inflammation of the aorta which results in stenosis, thrombosis and aneurysm formation. Half of the patients present with an initial systemic illness whereas the other 50% present with late-phase complications.
Two phases of the disease are classically described:
- pre-pulseless phase: characterized by non-specific systemic symptoms
- pulseless phase: presents with limb ischemia or renovascular hypertension
The initial systemic illness may include symptoms of malaise, fever, night sweats, weight loss and arthralgia. There is often anemia with raised inflammatory markers. This phase gradually resolves with the initiation of the chronic phase which is characterized by inflammatory and obliterative changes in the aorta and its branches. There are often reduced or absent peripheral pulses, giving rise to its alternative name of "pulseless disease".
Cardiac complications can occur in up to 60% of cases 9.
In some situations pulmonary arterial involvement can also occur 10-11
It has been classified based on location 3:
- type I: classic type involving solely the aortic arch branches: brachiocephalic trunk, carotid and subclavian arteries
- IIa: involvement of the aorta solely at its ascending portion and/or at the aortic arch +/- branches of the aortic arch
- IIb: involvement of the descending thoracic aorta +/- ascending or aortic arch + branches
- type III: involvement of the thoracic and abdominal aorta distal to the arch and its major branches, e.g. descending thoracic aorta + abdominal aorta +/- renal arteries
- type IV: sole involvement of the abdominal aorta and/or the renal arteries
- type V: generalized involvement of all aortic segments
Findings include 5:
- long, smooth, homogeneous and moderately echogenic circumferential thickening of the arterial wall may be present; on transverse section, this finding is termed as the 'macaroni sign' and is highly specific for Takayasu arteritis (in contrast, atherosclerotic plaque is non-homogeneous, often calcified with irregular walls and generally affects a short segment)
- vascular occlusion may be seen due to intimal thickening and/or secondary thrombus formation
- flow velocities depend on the level of occlusion
- aneurysms may be noted
- there may be a loss of pulsatility of the vessel
Findings include 2:
- wall thickening: active acute phase
- wall enhancement: active acute phase
- aortic valve disease: stenosis, regurgitation
- occlusion of major aortic branches
- aneurysmal dilatation of the aorta or its branches
- pseudoaneurysm formation
- diffuse narrowing distally (i.e. descending and abdominal aorta): in the late phase
The pulmonary arteries are also commonly involved, with the most common appearance being peripheral pruning.
Described features on CTCA include 7:
- stenosis of the coronary ostia: ~30%
- non-ostial coronary arterial stenoses: ~35%
- coronary arterial aneurysms: ~10%
- combination of stenosis and aneurysms ('string of pearls sign') 9
Treatment and prognosis
Treatment is with systemic steroids and judicious use of angioplasty. Corticosteroids can be used for initial treatment. Other medical options include methotrexate, cyclophosphamide, and cyclosporine. Percutaneous angioplasty and bypass surgery should only be considered when there is no acute inflammation.
Prognosis tends to be variable ranging from a rapidly progressive disease in some reaching a quiescent stage in others.
History and etymology
The condition is named after, a Japanese ophthalmologist, Mikito Takayasu (1860-1938) who initially described similar vascular features on the retina in a 22 year old female patient in 1908 12.
Takayasu arteritis has been given various other monickers over the years including pulseless disease, aortic arch syndrome, occlusive coagulant aortic syndrome, idiopathic medial aortopathy and obstructive productive arteritis 12,13.
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