Takayasus arteritis

Takayasu's arteritis is a term synonymous with pulseless disease, aortic arch arteritis or syndrome, or reversed coarctation. It is a chronic inflammatory disease of unknown etiology that affects the large arteries. The aorta and its main branches are primarily involved. Histological features include continuous or patchy panarteritis with granulomatous inflammation and infiltration by lymphocytes, plasma cells, histiocytes, and multinucleated giant cells.

Takayasu's arteritis is more common in Asia, although the disease has been described in all parts of the world. The female-to-male ratio is 8.5:1, and 80 per cent of patients are aged between 11 and 30 years. Initial or acute clinical features include fever, malaise, weight loss, arthralgias, and night sweats lasting 4 to 6 weeks. Chronic disease is the result of chronic ischemia of affected organs.

Pulmonary artery involvement is present in up to 50 per cent of all patients with Takayasu's arteritis. Pathological lesions are generally localized to medium and large pulmonary arteries. The histological abnormalities described above lead to pulmonary arterial occlusion and stenoses. A retrospective study of perfusion lung scans revealed abnormalities in 76 per cent of 120 patients with Takayasu's arteritis; initially, the changes appeared in the upper lobes and then progressed to involve arteries of the middle and lower lobes. In another study of 42 patients, in whom a respiratory problem was not clinically suspected, intravenous digital subtraction angiography showed involvement of the pulmonary artery in 14.3 per cent, even though the chest radiographs were abnormal in only two patients. A comparison study of 59 patients with temporal arteritis showed that, although chest radiographs were abnormal in 68 per cent, pulmonary angiography revealed arterial occlusions in 86 per cent. A poor correlation has been noted between angiographic changes and the results of spirometry and arterial blood gas analysis. Other thoracic complications described include aneurysms of pulmonary arteries. Takayasu arteritis presenting as idiopathic adult respiratory distress syndrome with a pathological diagnosis of acute interstitial pneumonia has been described.

Corticosteroid therapy results in symptomatic remission within days to weeks. Patients with significant pulmonary arterial occlusions may require surgical bypass. Death is usually the result of vascular complications such as rupture of an aneurysm, myocardial infarction, congestive cardiac failure, or cerebrovascular accident. Pulmonary involvement signifies a poor prognosis.

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