Heart Vessels (1992) Suppl. 7:3-5

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Introductory remarks for this special issue on Takayasu arteritis Fujio N u m a n o Department of Internal Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113 Japan

In 1905, Mikito Takayasu, Professor of Ophthalmology at Kanazawa University, reported at the Annual Meeting of the Japan Ophthalmology Society in Fukuoka a case of a 21-year-old woman who exhibited an interesting eyeground view characterized by coronary anastomosis. This was the first case of Takayasu arteritis Takayasu arteritis to be reported and in 1908 an abstract was published in the Proceedings of the Japan Ophthalmology Society [1]. As this abstract was written in Japanese, an English translation by Drs. Matsubara will be included as an Appendix to these remarks, for the convenience of researchers interested in this disease. At this meeting, Katsutomo Onishi, Professor of Ophthalmology at Kyushu University, and Tsurekichi Kagoshima, Professor of Ophthalmology at Kumamoto University, corroborated his presentation with their cases of eyegrounds showing the same features. Furthermore, they mentioned that the pulse could not be palpated in both radial arteries (Dr. Onishi) or in the left radial artery (Dr. Kagoshima). Soon afterwards, several cases were reported in Japan, including a report in 1939 by Yasuzo Shinmi who used the term Takayasu's disease for the first time [2]. Before the first presentation of this disease by Dr. Takayasu, a few eases describing no palpation of the radial arteries had been reported in the medical literature. For instance, in 1856 Savory reported the case of a 22-year-old woman whose radial and left carotid arteries could not be palpated. There was a feeble pulsation in the right carotid, and vision in the left eye was lost [3]. Later he reported findings following autopsy of this case in which he described irregular patches of an opaque yellowish deposit in aU branches of the aortic arch. The vessels became fibrous at a .few inches beyond the origin from the aorta for which he suggested a healed dissecting aneurysm of the aorta [41. In 1940, Kunio Ota, Department of Pathology,

Tokyo University, reported for the first time the systematic pathological survey of a 25-year-old female patient suffering from Takayasu arteritis, making clear this morbid condition was caused by vasculitis involving the aorta and its main branches, including pulmonary arterites. He stressed that Takayasu arteritis is panaortitis, involving the whole vessel wall, i.e., the intima, media, and adventitia. The report also concluded that changes in the eye-ground resulted in occlusion of the carotid artery [5]. Takayasu arteritis has been well known in foreign countries since Kentaro Shimizu and Keiji Sano, Professors of Surgery and Cerebrovascul Surgery at Tokyo University, introduced this disease in the English literature as pulseless disease. They described the characteristic clinical features of pulselessness, coronary anastomosis in retinat vasculature, and accentuated carotid sinus reflex as the triad of this morbid condition in 1948 [6]. In addition, an introductory paper on the disease by Caccamize and Whiteman in the American Heart Journal contributed to the spread of information on Takayasu arteritis into Western countries. Caccamize obtained resources from Kunio Okuda [7]. Ross and McKusick, after Frevig's paper reporting a 21-year-old woman with bilateral obliteration of the common carotid artery [8], summarized over 100 cases in which absent or diminished pulses in the arm and neck were observed, and they used the term "aortic arch syndrome" in which the cause of this morbid condition was due to stenoses or obstruction near the origin of the great vessels from the arch of the aorta..They described many cases.and classified them according to their causative factors, such as syphilic aortitis, atheromatosis, congenital anomalies, and trauma thrombophilia [9]. They also mentioned four similar cases in the literature, reported by Frevig as ù young feinale arteritis," pointing out that all these patients were young females and that they showed cerebral and occular manifestations.

4 In 1963, Hideo Ueda, Professor of Internal Medicine at Tokyo University, through studies of many cases of Takayasu arteritis, confirmed that it is due to aortitis involving the aorta, its main branches, pulmonary, and coronary arteritis and called it "pan aortitis syndrome." He suggested it could be induced by an autoimmune mechanism [10]. Afterwards, he changed this name to "aortitis syndrome" because of potential misunderstandings whereby "pan" would be taken to mean the whole area of the aorta. In 1975, the research committee of the Department of Health and Welfare in Japan proposed the use of "Takayasu arteritis" in mernory of the first reporter, Mikito Takayasu. The etiology of this morbid condition is still unclear. Nowadays there is widespread belief that it is a mulifactorial disease. This special issue is aimed at an international discussion of Takayasu arteritis because one of the characteristic features of this disease is "species differences." That is, young female patients with Takayasu arteritis are frequently encountered in Asian and South American countries, but there are very few in Western countries. Interesting questions include why such species difference occurs, and whether or not there are some differences in the clinical features or time courses among patients from Asian countries. Could hereditary factor(s) be postulated as playing a role in this morbid condition? These subjects should be clarified in order to ellucidate its etiology. We have already carried out one international project, the "Japan Korea eollaborative study on Takayasu arteritis in 1989 and 1990," through the support of the Department of Education in Japan. This research allowed the discussion two characteristic features of this disease, the difference in lesions of the aorta between Korean and Japanese patients and the high frequency of some HLA antigens. With this background, we will now tun a second project, "Comparative studies on Takayasu arteritis among Asian countries in 1991-1992." We hope this issue will contribute to progress in out project and to elucidating up the etiology of this disease.

Acknowledgements. In editing this issue, our coeditor, K.T. Lee, Professor of Pathology at George Washington University, passed away on April 30th 1992. I would like to express my sincere appreciation and respect for his warmhearted contributions and continuous encouragement.

F. Numano: Introductory remarks disturbance in September, 1904. This was associated with conjunctival hyperemia. She was relieved of the discomfort by some medication, but similar complaints returned in March 1905. There had been no previous history of serious illnesses. Ophthalmologic examination revealed retarded pupillary reaction with slight dilatation of the pupillae. The vasculature of the retina showed coronary anastomosis surrounding the papilla at a distance of 2 - 3 m m . Another annular vascular anastomosis was observed around the peripapillary lesion just mentioned. There was evidence of arteriovenous shunts. The inner vascular circle around the papillae was slightly elevated from the retinal surface. The two vascular circles and their branches revealed several nodular structures which changed their location day by day. The latter lesions were mainly related with the arteries. Hemorrhage was observed, but no sign of inflammation. The findings were essentially symmertric, but more prominent in the left eye. Sight: counting fingers at 0.5 m. There were no changes on urinary analysis, not any pathologic findings in other parts of the body. Iodine and potassium bromide were given per os, and saline was injected subconjunctivally. Soon the sight improved to distinction of fingers at 2.0m. Pilocarpine was given starting on June 11. In August, mydriasis occurred, the eye ball became harder in the left eye, and glaucoma followed. Cataract was noticed first in the right eye, and then, on July 14, the left eye was similarly affected. Due to certain circumstances in her family the patient left the clinic, but returned on July 21, at which time it was found that her sight had been severely damaged (counting fingers at 0.5 m on the right; at 1.0m on the left). In August her sight was poorer yet (counting fingers at 0.2m on the left). In view of the retinal lesions, operation for cataract was hopeless. However, the patient insisted upon undergoing such an operation. In spite of an uneventful operative procedure, her sight did not come back. Retinal detachment occurred on the left eye and the right eye became hypotonic. Her sight was completely lost. The patient was 21 years old, and apparently otherwise healthy. There was no indication of tuberculosis. The genesis of this disease is inexplicable to the author.

Appendix Discussion Takayasu M (1908) A case with peculiar lesions of the retinal central vessels. Acta Soc Ophthal Jpn 12:554555 (translated by Drs. Osamu and Akiko Matsubara) This peculiar observation was made in May 1905 on a woman. The patient first noticed bilateral visual

Dr. Ohnishi: "We have at present in out clinic a patient with quite a similar pathology. There are vascular circles with an'nular anastomosis in association with aneurysmatic lesions. Most remarkable is that the pulsation of the bilateral radial arteries is

F. Numano: Introductory remarks impalpable. The arms are cold. There is also mydriasis and cloudiness of the lens. After removal of the lenses, anterior chamber hemorrhage (hyphema) occurred and further observation of the retina is now impossible." Dr. Kagoshima: "I have also experienced a case without radial pulsation of the left arm. However, the arm was not cold. The patient lost her sight. Retinal lesions were not observed due to cataract. The other eye revealed infiltration and low vision." Dr. Abe: "Did it occur suddenly?" Dr. Takayasu: "The onset of the disease is not clear. According to the patient's statement, her first trouble was cured by a certain treatment. A n inquiry to the doctor who treated her first should be made."

References 1. Takayasu M (1908) A case with peculiar changes of the retinal central vessels (in Japanese). Acta Soc Ophthal Jpn 12:554-555 2. Shinmi Y (1942) A case of Takayasu's disease (in Japanese). Sogo Gannka 36:1404-1410

5 3. Savory W (1856) On a case of a young woman in whom the main arteries of both upper extremities and of the left side of the neck were throughout completely obliterated. Lancet April 5:373 4. Savory W (1856) On a case of a young woman in whom the main arteries of both upper extremities and of the left side of the neck were throughout completely obliterated. Med Chir Trans London 39:205-219 5. Ota K (1940) Ein seltener Fall von beiderseitigem carotis - subclavia verschluss (Ein Beitrag zur Pathologie der Anastomosis peripapillaris des Auges mit Fehlendem Radialpuls.) Soc Path Jap 30:680-690 6. Shimizu K, Sano K (1951) Pulseless disease. J Néuropath clin 1:37 7. Caccamze WC, Whiteman JF (1952) Pulseless disease: A preliminary case report. Am Heart J 44:629-633 8. Frovig AG (1946) Bilateral obliteration of the common artery. Acta psychiat et neurol, Supp. 39 9. Ross RS, McKusick VA (1953) Aortic arch syndromes diminished or absent pulses in arteries arising from the arch of the aorta. AMA Int Med 92:701-740 10. Ueda H (1963) Aortic arch syndrome with special references to pulseless disease and its variants Jpn Heart J 4:224-234 11. Judge RD, Currier RD, Gracie WA, Figley MM (1962) Takayasu arteritis and the aortic arch syndrome. Am J Med 32:379-392 12. Nasu T (1975) Takayasu's truncoarteritis in Japan: A statistical obervation of 76 autopsy cases. Pathol Microbiol 43:140-146

Introductory remarks for this special issue on Takayasu arteritis.

Heart Vessels (1992) Suppl. 7:3-5 Heart aWessels © Springer-Verlag1992 Introductory remarks for this special issue on Takayasu arteritis Fujio N u...
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