In 1830, Rokushu Yamamoto described a peculiar condition of a 45 year old man in his private practice record. The said man initially came to Yamamoto with a complain of high grade fever and a year later, while examining the man, Yamamoto found that there were no palpable pulses on the patient’s upper limbs and carotid. Later on, the patient lost weight, had difficulty in breathing and the aforementioned problems worsened with time before he eventually died many years after.
Nearly a century later, during the 12th Annual Meeting of The Japanese Opthalmology Society, Professor Mikito Takayasu presented a case of a 21-year old young lady whose optic fundi exhibited peculiar “wreathlike” appearance of the blood vessels in the back of the eye (retina), a coronary arteriovenous anastomosis. At the same meeting, Dr Onishi and Dr Kagosha each contributed a case of their own with similar fundus finding. However, in addition, both pointed out that no radial pulses could be palpated in their respective patients.
The unveiling autopsy
The effort to unveil this mystery disease reached a new milestone in 1920 when a postmortem on a deceased patient with this disease was done. What was found? They found panarteritis involving all layers of the artery, causing constriction of the affected vessel. Such constriction reduces the blood flow and the flow of oxygenated blood and when the affected vessel is the the vessel around the cervical area supplying the head, reduction in oxygen supply to the head occur.
This explains what Takayasu saw in the retina of his patient – the formation of the anastomosis on the retina is a form of adaptive angiogenesis to maintain enough oxygen in the area. Finally, in 1951, Dr Shimizu and Dr Sano summarised the clinical features of Takayasu’s arteritis in an English journal, under the name of the “pulseless disease.”
Current facts on Takayasu’s Disease.
Nowadays, this disease has been called by a few names such as Takayasu’s disease, “aortic arch syndrome,” Takayasu’s arteritis and the most enigmatic of all, the “Pulseless Disease.” Regardless of what name you choose to call it, this condition is characterised by inflammation of large vessels such as the aorta, the arch of aorta, the pulmonary artery and the subclavian artery.
Epidemiologically, Takayasu arteritis is rare, but most commonly seen in Japan, South East Asia, India, and Mexico. According to a study of North American patients, it was found that the incidence to be 2.6/million/year. Most commonly, it affects young or middle-age women of Asian descent and females are about 8–9 times more likely to be affected than males.
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Review Date: March 13, 2019 | Last Modified: November 15, 2019