Takayasu arteritis: clinical features and management: report of 272 cases

BACKGROUND: Takayasu's arteritis is a condition of unknown aetiology with an unpredictable natural history. Most of the literature available has originated from Asia, with a few contributions from Africa where the pattern of the disease may be different. This is a single institution's experience review. METHODS: Data were obtained retrospectively from the angiographic and medical records of patients treated at Groote Schuur Hospital over the period 1952-2002. The criteria for inclusion were those proposed by the Aortitis Syndrome Research Committee of Japan and the American College of Rheumatology. RESULTS: Two hundred and seventy-two patients were identified. The mean age at presentation was 25 years (range 14-66 years) and 75% were female. Only 8% were Caucasian. Hypertension was the most common presentation (77%) and was usually a consequence of renal artery stenosis or aortic coarctation. Cardiac failure was the most common problem. Cerebrovascular symptoms were recorded in 20%. Convincing evidence of tuberculosis was present in 20%. The entire aorta was involved in 70% of cases. Thirty per cent had aortic bifurcation involvement. Occlusions were noted in 93% and aneurysms in 46%. Vascular reconstruction was performed on 115 occasions in 99 patients, with an operative mortality of 4%. Cardiac failure was the usual cause of death. One hundred and six patients (39%) were followed for a minimum of 5 years. No further progression of disease was noted in 70 patients. CONCLUSION: The natural history and prognosis of Takayasu's arteritis still remain poorly defined.