TAKAYASU DISEASE
Rarely, other features of connective tissue diseases, e.g. arthritis, weight loss, erythema nodosum rash, etc. may be present.
Diagnosis is suspected clinically in presence of unexplained hypertension and confirmed on angiography to demonstrate site of obstruction (Table 24.14).
In India, all cases should also be investigated for tuberculosis, though the cause-effect relationship is uncertain.Treatment includes: (a) anti-hypertensive therapy, (b) non-invasive balloon/stent dilatation or surgical repair of obstructive vascular lesions, and (c) immunosuppressive therapy with steroids, methotrexate or cyclophosphamide in progressive disease. Anti-tubercular therapy has been used in cases with suspected tubercular etiology, though it is unlikely to prevent progression of vascular lesions. Prognosis depends on severity at diagnosis and further progression of disease, with 5-year mortality of ~5%, usually due to aneurysmal rupture.
BIBLIOGRAPHY
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