Heart Vessels (1992) Suppl. 7:6-10
Heart andVessel s © Springer-Verlag1992
An overview on Takayasu arteritis Morie Sekiguchi and Jun-ichi Suzuki First Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390 Japan
Summary. Takayasu arteritis is a non-specific inflammatory diseäse of unknown etiology. It was first recognized as having a peculiar wreath-like arteriovenous anastomosis around the papillae of the retina by a Japanese ophthalmologist, Dr. M. Takayasu in 1908. A Japanese research committee reported more than 5,000 cases. Fo? a supplement issue on Takayasu arteritis, this brief overview article has been written as an introduction to the disease. Key words: Takayasu arteritis - Overview - Clinical Aspects - History
Introduction Takayasu arteritis is a non-specific inflammatory disease of the arteries with unknown etiology [1-39]. It involves large arteries such as the aorta and its branches, including the common carotid, subclavian, pulmonary, and eoronary arteries. It is observed clinically as obstructive symptoms due to stenosis and thrombus formation on the intima of the arteries. Because of these eharacteristics, it is also known as "pulseless disease" [8, 9, 11, 15, 17-19, 22]. This disease was reported in 1908 by an ophthalmologist, Mikito Takayasu (Fig. 1), a professor of Ophthalmology at Kanazawa University, Japan [3]. He noticed a peculiar wreath-like arteriovenous anastomosis around the papillae. In 1952, this disease was introduced as "Takayasu's disease" by Caccamise and Whitman [11]. The name pulseless disease was given by Professors Shimizu and Sano [8], who were neurosurgeons. Since this disease was frequently
Address correspondence to: M. Sekiguchi
observed in Japan, a research committee, sponsored by the Japanese government's Ministry of Health and Welfare, has been studying this disease and gave it the name of "Takayasu's arteritis." [39] A report of the committee showed the number of patients recorded during 1973-75 amounted to 2,148, of which 1,909 (89%) were female. Also, the report from 1982-84 revealed 2,606 patients, and of these, 90% were female [40]. The etiology of this disease is still unknown [39]. A relation with tuberculosis (TB) has often been speculated because the pathology looks like that of TB. However, Takayasu arteritis is prevalent among those without any other evidence of TB. Therefore, a direct relation with TB has been denied. However, this relationship is still considered important in India because of a high linkage with TB. The most important etiological agent in this disease is the autoimmune mechanism [41-50]. In 1964, Ueda's research group [46] detected an antiaortic antibody among patients with this disease. It was detected by complement fixation reaction and also by the use of immunoflourescence antibody method. This disease was experimentally reproduced in rabbits with the repeated sensitization with the extract from the aortic wall of the patients with this disease. The reason for the high prevalence in women is still obscure. This disease is also reported to be prevalent in India, Thailand, China, Korea, and Israel. Also it is reported in South American countries such as Mexico [39, 51-53]. Deutsch et al. [32] reported that 22 patients in Israel with Takayasu arteritis were all Sephradis, or those of the Jewish nation with Spanish traits. On the contrary, Ashkenazis (Jewish with German traits) did not show any prevalence of this disease. Therefore, racial difference is a preceding characteristic. These facts suggest that the hereditary factor is important in the development of this disease. Human leukocyte antigen (HLA) is considered an important factor. Numano et al. pointed out that HLA
M. Sekiguchi and J. Suzuki: An overview
Fig. 1. Dr. Mikito Takayasu (1860-1938)
AW24-BW52-DW12 haplotype patients are prone to developing rapid progress of the disease and also show resistance to corticosteroid therapy [54, 55].
Pathology The pathology of this disease is most prevalent at the aortic arch area and the name trunco-arteritis has been used [56-62]. Figure 2 shows the distribution of the arterial lesions in an autopsied case with this disease [561. Histopathlogically, the inflammatory process is observed at the media of the arteries and exterlds to the adventia and intima. The cell infiltrations are composed of lymphocytes and epithelioid cells. The most characteristic lesion is the destruction of the elastic fibers of the media and is associated with the atrophy, disappearance, and replacement with fibrosis of the smooth muscle cells of the media. Calcification in those areas is also characteristic. Thickening of the intima is also commonly seen. However, it is presumed to be occurring as the secondary phenomenon due to the impairment of the transintimal and/of trans-
7
Fig. 2. Schema presenting the arterial lesions. AO, aorta; CA, coronary artery; PA, pulmonary artery; rCC, right common carotid artery; ICC, left common carotid artery; rSA, right subclavian artery; rVA, right vertebral artery; rlM, right internal mammary artery; ISA, left subclavian artery; IVA, left vertebral artery; llM, left internal mammary artery; D, diaphragm; dotted area, intirna; vertical lined area, media; hatched area, adventitia. (From Takeda et al. [56] with permission)
adventitial blood supply within the arterial wall itself [56]. When the arteritis develops rapidly, the artery becomes dilated and forms an aneurysm and aortic valve regurgitation occurs [56-62]. According to the site of this disease, it is classified into aortic arch types, the thoraco-abdominal type and the diffused type. The aortic arch type is most frequent [40, 63].
Diagnosis Subjective symptoms of this disease are variable [27, 40]. General fatigue, vertigo, and cold extremities are confused with the indefinite syndrome among women. An unpalpable pulse is the first opportunity to detect this disease so it is important to palpate both radial arteries at the time of an initial examination. When unexplainable calcification is found in the great arteries or diastolic murmur which suggests the presencé of aortic regurgitation or the presence of hyper-
8 Table 1. Guidelines for making a clinical diagnosis of Takayasu arteritis 1. Symptoms (1) Cerebral ischemia: vertigo (especially when looking upward), fainting spells, visual disturbance (especially at direct sunshine) (2) Ischemia of the extremities: cold fingers, easy fatiguability of the upper extremities (3) Stenosis of the aorta or renal arteries: headache, vertigo, shortness of breath, which are considered due to hypertension (4) Generalized symptoms: slight fever may be recognized at the onset of the disease 2. Important findings for diagnosis (1) Abnormalities of the pulse of the upper extremities (weak or diminution and/or right/left difference of the radial pulse) (2) Abnormalities of the pulse of the lower extremities (accentuation or decrease of the pulse) (3) Vascular murmur in the arteries of the heck, back, or abdomen (4) Ophthalmologic abnormalities 3. Abnormalities in laboratory examinations (1) Increased erythrocytes sedimentation rate (2) Positive C-reactive protein (3) Increase in gamma-globulin levels in the serum 4. Important diagnostic points (1) Prevalent in young women (2) Final clinical diagnosis can be made by an aortography 5. Differential diagnosis to be made: Buerger's disease, arteriosclerosis, collagen disease, congenital vascular abnormalities Translated into English by the authors from a report of the committee of Takayasu arteritis of the Ministry of Health and Welfare of Japan
M. Sekiguchi and J. Suzuki: An overview 1,364). Direct cause of death in this disease in 34 cases reported by Koide in 1986 was congestive heart failure (9 cases, 26%), followed by myocardial infarction (6 cases, 18%) and rupturing of the aortic aneurysm (3 cases, 9%) [40].
Treatment As the etiology of this disease is not known, the treatment is a symptomatic one [40, 69, 70]. Corticosteroid therapy is effective for the acute onset of this disease [69, 70]. The Japanese research committee suggested that those patients who are H L A positive need higher dosages and need to be watchful for the recurrence of this disease. Because the autoimmune process was considered as an etiological agent, immunosuppressive therapy was thought to be effective. However, this is no longer the case. Antiplatelet therapy is considered reasonable to prevent the progress of the disease. Balloon angioplasty, surgical intervention for the correction of the vascular stenosis, aortic valve replacement for aortic root dilatation, and aortocoronary bypass surgery for the ostial stenosis of the coronary arteries are effective [71-73].
Acknowledgements. This article was prepared for the purpose of introducing a brief outline of Takayasu arteritis in an issue of a proceedings of this disease. A picture of Dr. Takayasu was kindly provided by Dr. F. Numano.
References tension, increased sedimentation rate are important checkpoints for establishing the diagnosis of this disease. In Table 1, a diagnostic guideline for this disease is shown.
Differential Diagnosis Arteriosclerotic cardiovascular disease, congenital vascular anomalies such as aortic stenosis or subclavian artery insufficiency, syphyllitic aortitis, annuloaortic ectasia due to cystic medial necrosis, stenosis of the renal arteries, tuberculosis, collagen disease, and Buerger's disease are to be differentiated.
Prognosis Prognosis of this disease is favorable [40, 64-68]. Japanese statistics for 1973-75 reveal that the death rate was only 1.9% (26 out of 1,374). A n o t h e r survey from 1982-84 revealed a death rate of 2.6% (36 out of
1. Savory DS (1856) A case of a young woman in whom the main arteries of both upper extremities and of the left side of neck were throughout completely obliterated. Med Chir Tr 39:205-219 2. Kussmaul A (1872) Zwei Fälle von spontaner allmählicher verschliessung grosser Halsarterienstamme. Dtsch Klin 24:461-465 3. Takayasu M (1908) Case with unusual changes of the central vessels in the retina (in Japanese). Acta Soc Ophthal Jap 12:554-555 4. Beneke R (1925) Ein eigentümlicher Fall schwieliger Aortitis. Virchow Arch [A] 254:723-733 5. Harbitz F (1926) Bilateral carotid arteritis. Arch Path Lab Med 1:499-510 6. Sato T (1938) Ein Seltener Fall von arteriellen Obliteration. Klin Wochenschr 17:1154-1157 7. Oota K (1940) Ein seltener Fall von beiderseitigem Carotis-Subclaviaverschlues. (Ein Beitrag zur Pathologie der anastomosis peripapillaris des Auges mit Fehlendem Radial-plus) Trans Soc Pathol Jpn 30:680-690 8. Shimizu K, Sano K (1948) Pulseless disease (in Japanese). Clin Surg (Tokyo) 3:377-396. 9. Shimizu K, Sano K (1951) Pulseless disease. J Neuropath Clin Neurol 1:37-47 10. Frovig AG, Locken AC (1951) Syndrome of obliteration of the arterial branches of the aortic arch due to arteritis. Acta Psychiatr Neurol Scand 26:313-337
M. Sekiguchi and J. Suzuki: An overview 11. Caccamise WC, Whitman JF (1952) Pulseless disease. Preliminary case report. Am Heart J 44:629-633 12. Ross RS, McKusick VA (1958) Diminished or absent pulses in arteries arising from the arch of the aorta. Aortic arch syndrome. Arch Intern Med 92:701-740 13. Gottsegen G, Szäm I (1956) Über eine eigenartige, unter dem Bilde des brachiozephalischen Arterienverschlusses verlaufende Gefasserkrankung. Z Kreislaufforsch 45:196- 202 14. Koszewski B J, Hubbard TF (1957) Pulseless disease due to brachial arteritis. Circulation 16:406-410 15. Birke G, Ejrup B, Olhagen E (1957) Pulseless disease A clinical analysis of 10 cases. Angiology 8:433-453 16. Correa P, Araujo J (1958) Arteritis of the aorta in young women. Am J Clin Path 29:560-568 17. Lessof MH, Glynn LE (1959) Pulseless syndrome. Lancet 1:799-801 18. Stoyanoff PC (1959) Case of pulseless disease. Takayasu's syndrome. Acta Med Scand 163:167-168 19. Thurlbeck WM, Currens JH (1959) Aortic arch syndrome (pulseless disease). Report of ten cases with three autopsies. Circulation 19:499-510 20. Basu AK (1961) Occlusive disease of the aorta and its main branches. Br J Surg 49:148-156 21. Inada K, Shimizu H, Yokoyama T (1962) Pulseless disease and atypical coarctation of the aorta with special reference to their genesis. Surgery 52:433-443 22. Inada K, Shimizu H, Kobayashi I, Ishiai S, Kawamoto S (1962) Pulseless disease and atypical coarctation of the aorta. Arch Surg 84:306-311 23. Judge RD, Currier RD, Gracie WA, Fingley MM (1962) Takayasu's arteritis and the aortic arch syndrome. Am J Med 32:379-392 24. Hong CY (1963) Pulseless disease with hypertension. Primary arteritis causing occlusion of the left subclavian artery, coarctation of the abdominal aorta and bilateral sclerosis of renal arteries. J Korea Med Assoc 6: 1161-1167 25. Sen PK, Kinare SG, Engineer SD, Parulkar GB (1963) The middle aortic syndrome. Br Heart J 35:610-618 26. Roberts WC, Wibin EA (1966) Idiopathic panaortitis, supra-aortic arteritis, granulomatous myocarditis and pericarditis. Am J Med 41:453-461 27. Nakao K, Ikeda M, Kimata S, Niitani H, Miyahara M, Ishimi Z, Hashiba K, Takeda Y, Ozawa T, Matsushita S, Kuramochi M (1967) Takayasu's arteritis. Clinical report of eighty-four cases and immunological studies of seven cases. Circulation 35:1141-1155 28. Vinijchaikul K (1967) Primary arteritis of the aorta and its main branches (Takayasu's arteriopathy). Am J Med 43:15-27 29. Mufioz N, Correa P (1970) Arteritis of the aorta and its major branches. Am Heart J 80:319-328 30. Reddy GR, Rao NR, Reddy MR et al. (1970) Pathology of pulseless disease. Pathol Microbiol (Basel) 34:10-20 31. Sen PK, Einare SG, Kelkar MD, Parulkar GB (1973) Non-specific aorto-arteritis. A monograph based on a study of 101 cases. McGraw-Hill, Bombay, New Delhi 32. Deutsch V, Wexler L, Deutsch H (1974) Takayasu's arteritis. An angiographic study with remarks on ethnic distribution in Israel. Am J Roentgenol 122:13-28 33. Lupi-Herrera E, Sanchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE (1977) Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 93: 94-103 34. Wiggelinkhuizen J, Cremin BJ (1976) Takayasu arteritis and renovascular hypertension in children. Pediatrics 62:209-217
9 35. Abe K, Miyazaki S (1982) Clinical aspect of aortitis syndrome with special reference to the relation between prognosis and hypertension. Jpn Circ J 46:190-193 36. Fiessinger JN, Tawfik-Taher S, Capron L, Laurian C, Cormier JM, Camilleri JP, Housset E (1982) Maladie de Takayasu. Criteres diagnostiques. Nouv Presse Med 11:583-586 37. Wong VCW, Wand RYC, Tse TF (1983) Pregnancy and Takayasu's arteritis. Am J Med 75:597-601 38. Liu Yu-Qing, Du Jia-Hui (1984) Aorto-arteritis. A collective angiographic experience in 244 cases. Int Angio 3:487-497 39. Numano F, Lee KT, Hong YC (eds) (1992) Takayasu arteritis 1992. Heart Vessels Suppl 7 40. Koide K (1992) Takayasu arteritis in Japan. Heart Vessels Suppl 7:48-54 41. Pokoruny J, Je~kovh Z (1962) Significance of immunological studies in peripheral obliterating vascular diseases. Circ Res 11:961-965 42. Hirch MS, Aikat BK, Basu AK (1964) Takayasu's arteritis. Report of 5 cases with immunologic studies. Bull John Hopkins Hosp 115:29-64 43. Ikeda M (1966) Immunologic studies on Takayasu's arteritis. Jpn Circ J 30:87-89 44. Ito I (1966) Aortitis syndrome with reference to detection of antiaorta antibodies from patients' sera. Jpn Circ J 30:75-78 45. Sano K, Saito I (1966) Immunological atudies of pulseless disease. Neurol Med Chirurg 8:28-39 46. Ueda H, Saito Y, Ito I, Yamaguchi H, Sugiura M, Morooka S (1967) Immunological studies of aortitis syndrome. Jpn Heart J 8:4-18 47. Ito I, Saito Y, Nonaka Y (1975) Immunological aspects of aortitis syndrome. Jpn Circ J 39:458-462 48. Hall S, Nelson AM (1986) Takayasu's arteritis and juvenile rheumatoid arthritis. J Rheumatol 13:431433 49. Scott D, Salmon MI Scott DL, Blann A, Bacon PA, Walton KW, Oakland CD, Slaney GF (1986) Takayasu's arteritis. A pathogenetic role for cytotoxic T lymphocytes. Clin Rheumatol 5:517-522 50. Sakhuja V, Gupta KL, Bhasin DK, Malik N, Chugh KS (1990) Takayasu's arteritis associated with u~cerative colitis. Gut 31:831-833 51. Ask-Upmark E (1954) On the pulseless disease outside of Japan. Acta Med Scand 149:161-178 52. Isaacson C (1961) An idiopathic aortitis in young Africans. J Pathol Bact 81:69-79 53. Lee K, Sohn K, Hong C, Kang S, Berg K (1967) primary arteritis pulseless (pulseless disease) in Korean children. Acta Paediatr Scand 56:526-536 54. Numano F, Isohisa I, Maezawa H, Juji T (1979) HLA antigens in Takayasu disease. Am Heart J 88:153-159 55. Isohisa I, Numano F, Maezawa H, Sasazuki T (1982) Hereditary factors in Takayasu's disease. Angiology 33:98-104 56. Takeda F, Ooneda G, Suto K, Suto H, Kubota K (1961) Pulseless disease. Report of an autopsy case, with special reference to the morphogenesis of the arterial lesions. Gunma J Med Sci 10:27-45 57. Nasu T (1983) Pathology of pulseless disease. A systemic study and clinical review of twenty-one autopsy cases reported in Japan. Angiology 14:225-242 58. Boström K, Hassler O (1965) Takayasu's disease. Postmortem examination of a previously published case. Acta Med Scand 178:537-542 59. Font RL, Naumann G (1969) Ocular histopathology in pulseless disease. Arch Ophthal 82:784-788
10 60. Shionoya S, Griss P (1969) Zur Pathogenese der Takayasu-Krankheit. Arthusphenomen an den Vasa Vasorum. Virchows Arch [A] 348:269-280 61. Nasu T (1976) Takayasu's truncoarteritis in Japan. A statistical observation of 76 autopsy cases. Pathol Microbiol 43:140-146 62. Cipriano PR, Silverman JF, Perloth MG, Gripp RB, Wexler L (1977) Coronary arterial narrowing in Takayasu's arteritis. Am J Cardiol 39:744-750 63. Lande A, Rossi P (1975) The value of total aortography in the diagnosis of Takayasu's arteritis. Radiology 114: 287-297 64. Strachan RW (1964) The natural history of Takayasu's arteriopathy. Q J Med 33:57-69 65. Kinare SG (1970) Aortitis in early life in India and its association with tuberculosis. J Pathol 100:69-76 66. Ishikawa K (1978) Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation 57:27-35 67. Ishikawa K (1981) Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu's disease). Am J Cardiol 47:1026-1032
M. Sekiguchi and J. Suzuki: An overview 68. Ishikawa K, Matsuura S (1982) Occlusive thromboaortopathy (Takayasu's disease) and pregnancy. Clinical course and management of 33 pregnancies and deliveries. Am J Cardiol 50:1293-1300 69. Hayashi K et al. (1986) Takayasu's arteritis. Decrease in aortic wall thickening following steroid therapy, documented by CT. Br J Radiol 59:281-283 70. Ishikawa K, Yonekawa Y (1987) Regression of carotid stenoses after corticosteroidd therapy in occlusive thromboaortopathy (Takayasu's disease). Stroke 18: 677- 679 71. Dong Z et al. (1987) Percutaneous transluminal angioplasty for renovascular hypertension in arteritis. Experience in China. Radiology 162:477-479 72. Seguchi M, Hino Y, Aiba S, Yasukohchi S, Momma K, Takao A, Endo M (1990) Ostial stenosis of the left coronary artery as a sole clinical manifestation of Takayasu's arteritis. A possible cause of unexpected sudden death. Heart Vessels 5:188-191 73. Suzuki A, Amano J, Tanaka H, Sakamoto T, Sunamori M (1989) Surgical consideration of aortitis involving the aortic root. Circulation 80 Suppl 1:222-232
Heart andVessel s © Springer-Verlag1992
An overview on Takayasu arteritis Morie Sekiguchi and Jun-ichi Suzuki First Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390 Japan
Summary. Takayasu arteritis is a non-specific inflammatory diseäse of unknown etiology. It was first recognized as having a peculiar wreath-like arteriovenous anastomosis around the papillae of the retina by a Japanese ophthalmologist, Dr. M. Takayasu in 1908. A Japanese research committee reported more than 5,000 cases. Fo? a supplement issue on Takayasu arteritis, this brief overview article has been written as an introduction to the disease. Key words: Takayasu arteritis - Overview - Clinical Aspects - History
Introduction Takayasu arteritis is a non-specific inflammatory disease of the arteries with unknown etiology [1-39]. It involves large arteries such as the aorta and its branches, including the common carotid, subclavian, pulmonary, and eoronary arteries. It is observed clinically as obstructive symptoms due to stenosis and thrombus formation on the intima of the arteries. Because of these eharacteristics, it is also known as "pulseless disease" [8, 9, 11, 15, 17-19, 22]. This disease was reported in 1908 by an ophthalmologist, Mikito Takayasu (Fig. 1), a professor of Ophthalmology at Kanazawa University, Japan [3]. He noticed a peculiar wreath-like arteriovenous anastomosis around the papillae. In 1952, this disease was introduced as "Takayasu's disease" by Caccamise and Whitman [11]. The name pulseless disease was given by Professors Shimizu and Sano [8], who were neurosurgeons. Since this disease was frequently
Address correspondence to: M. Sekiguchi
observed in Japan, a research committee, sponsored by the Japanese government's Ministry of Health and Welfare, has been studying this disease and gave it the name of "Takayasu's arteritis." [39] A report of the committee showed the number of patients recorded during 1973-75 amounted to 2,148, of which 1,909 (89%) were female. Also, the report from 1982-84 revealed 2,606 patients, and of these, 90% were female [40]. The etiology of this disease is still unknown [39]. A relation with tuberculosis (TB) has often been speculated because the pathology looks like that of TB. However, Takayasu arteritis is prevalent among those without any other evidence of TB. Therefore, a direct relation with TB has been denied. However, this relationship is still considered important in India because of a high linkage with TB. The most important etiological agent in this disease is the autoimmune mechanism [41-50]. In 1964, Ueda's research group [46] detected an antiaortic antibody among patients with this disease. It was detected by complement fixation reaction and also by the use of immunoflourescence antibody method. This disease was experimentally reproduced in rabbits with the repeated sensitization with the extract from the aortic wall of the patients with this disease. The reason for the high prevalence in women is still obscure. This disease is also reported to be prevalent in India, Thailand, China, Korea, and Israel. Also it is reported in South American countries such as Mexico [39, 51-53]. Deutsch et al. [32] reported that 22 patients in Israel with Takayasu arteritis were all Sephradis, or those of the Jewish nation with Spanish traits. On the contrary, Ashkenazis (Jewish with German traits) did not show any prevalence of this disease. Therefore, racial difference is a preceding characteristic. These facts suggest that the hereditary factor is important in the development of this disease. Human leukocyte antigen (HLA) is considered an important factor. Numano et al. pointed out that HLA
M. Sekiguchi and J. Suzuki: An overview
Fig. 1. Dr. Mikito Takayasu (1860-1938)
AW24-BW52-DW12 haplotype patients are prone to developing rapid progress of the disease and also show resistance to corticosteroid therapy [54, 55].
Pathology The pathology of this disease is most prevalent at the aortic arch area and the name trunco-arteritis has been used [56-62]. Figure 2 shows the distribution of the arterial lesions in an autopsied case with this disease [561. Histopathlogically, the inflammatory process is observed at the media of the arteries and exterlds to the adventia and intima. The cell infiltrations are composed of lymphocytes and epithelioid cells. The most characteristic lesion is the destruction of the elastic fibers of the media and is associated with the atrophy, disappearance, and replacement with fibrosis of the smooth muscle cells of the media. Calcification in those areas is also characteristic. Thickening of the intima is also commonly seen. However, it is presumed to be occurring as the secondary phenomenon due to the impairment of the transintimal and/of trans-
7
Fig. 2. Schema presenting the arterial lesions. AO, aorta; CA, coronary artery; PA, pulmonary artery; rCC, right common carotid artery; ICC, left common carotid artery; rSA, right subclavian artery; rVA, right vertebral artery; rlM, right internal mammary artery; ISA, left subclavian artery; IVA, left vertebral artery; llM, left internal mammary artery; D, diaphragm; dotted area, intirna; vertical lined area, media; hatched area, adventitia. (From Takeda et al. [56] with permission)
adventitial blood supply within the arterial wall itself [56]. When the arteritis develops rapidly, the artery becomes dilated and forms an aneurysm and aortic valve regurgitation occurs [56-62]. According to the site of this disease, it is classified into aortic arch types, the thoraco-abdominal type and the diffused type. The aortic arch type is most frequent [40, 63].
Diagnosis Subjective symptoms of this disease are variable [27, 40]. General fatigue, vertigo, and cold extremities are confused with the indefinite syndrome among women. An unpalpable pulse is the first opportunity to detect this disease so it is important to palpate both radial arteries at the time of an initial examination. When unexplainable calcification is found in the great arteries or diastolic murmur which suggests the presencé of aortic regurgitation or the presence of hyper-
8 Table 1. Guidelines for making a clinical diagnosis of Takayasu arteritis 1. Symptoms (1) Cerebral ischemia: vertigo (especially when looking upward), fainting spells, visual disturbance (especially at direct sunshine) (2) Ischemia of the extremities: cold fingers, easy fatiguability of the upper extremities (3) Stenosis of the aorta or renal arteries: headache, vertigo, shortness of breath, which are considered due to hypertension (4) Generalized symptoms: slight fever may be recognized at the onset of the disease 2. Important findings for diagnosis (1) Abnormalities of the pulse of the upper extremities (weak or diminution and/or right/left difference of the radial pulse) (2) Abnormalities of the pulse of the lower extremities (accentuation or decrease of the pulse) (3) Vascular murmur in the arteries of the heck, back, or abdomen (4) Ophthalmologic abnormalities 3. Abnormalities in laboratory examinations (1) Increased erythrocytes sedimentation rate (2) Positive C-reactive protein (3) Increase in gamma-globulin levels in the serum 4. Important diagnostic points (1) Prevalent in young women (2) Final clinical diagnosis can be made by an aortography 5. Differential diagnosis to be made: Buerger's disease, arteriosclerosis, collagen disease, congenital vascular abnormalities Translated into English by the authors from a report of the committee of Takayasu arteritis of the Ministry of Health and Welfare of Japan
M. Sekiguchi and J. Suzuki: An overview 1,364). Direct cause of death in this disease in 34 cases reported by Koide in 1986 was congestive heart failure (9 cases, 26%), followed by myocardial infarction (6 cases, 18%) and rupturing of the aortic aneurysm (3 cases, 9%) [40].
Treatment As the etiology of this disease is not known, the treatment is a symptomatic one [40, 69, 70]. Corticosteroid therapy is effective for the acute onset of this disease [69, 70]. The Japanese research committee suggested that those patients who are H L A positive need higher dosages and need to be watchful for the recurrence of this disease. Because the autoimmune process was considered as an etiological agent, immunosuppressive therapy was thought to be effective. However, this is no longer the case. Antiplatelet therapy is considered reasonable to prevent the progress of the disease. Balloon angioplasty, surgical intervention for the correction of the vascular stenosis, aortic valve replacement for aortic root dilatation, and aortocoronary bypass surgery for the ostial stenosis of the coronary arteries are effective [71-73].
Acknowledgements. This article was prepared for the purpose of introducing a brief outline of Takayasu arteritis in an issue of a proceedings of this disease. A picture of Dr. Takayasu was kindly provided by Dr. F. Numano.
References tension, increased sedimentation rate are important checkpoints for establishing the diagnosis of this disease. In Table 1, a diagnostic guideline for this disease is shown.
Differential Diagnosis Arteriosclerotic cardiovascular disease, congenital vascular anomalies such as aortic stenosis or subclavian artery insufficiency, syphyllitic aortitis, annuloaortic ectasia due to cystic medial necrosis, stenosis of the renal arteries, tuberculosis, collagen disease, and Buerger's disease are to be differentiated.
Prognosis Prognosis of this disease is favorable [40, 64-68]. Japanese statistics for 1973-75 reveal that the death rate was only 1.9% (26 out of 1,374). A n o t h e r survey from 1982-84 revealed a death rate of 2.6% (36 out of
1. Savory DS (1856) A case of a young woman in whom the main arteries of both upper extremities and of the left side of neck were throughout completely obliterated. Med Chir Tr 39:205-219 2. Kussmaul A (1872) Zwei Fälle von spontaner allmählicher verschliessung grosser Halsarterienstamme. Dtsch Klin 24:461-465 3. Takayasu M (1908) Case with unusual changes of the central vessels in the retina (in Japanese). Acta Soc Ophthal Jap 12:554-555 4. Beneke R (1925) Ein eigentümlicher Fall schwieliger Aortitis. Virchow Arch [A] 254:723-733 5. Harbitz F (1926) Bilateral carotid arteritis. Arch Path Lab Med 1:499-510 6. Sato T (1938) Ein Seltener Fall von arteriellen Obliteration. Klin Wochenschr 17:1154-1157 7. Oota K (1940) Ein seltener Fall von beiderseitigem Carotis-Subclaviaverschlues. (Ein Beitrag zur Pathologie der anastomosis peripapillaris des Auges mit Fehlendem Radial-plus) Trans Soc Pathol Jpn 30:680-690 8. Shimizu K, Sano K (1948) Pulseless disease (in Japanese). Clin Surg (Tokyo) 3:377-396. 9. Shimizu K, Sano K (1951) Pulseless disease. J Neuropath Clin Neurol 1:37-47 10. Frovig AG, Locken AC (1951) Syndrome of obliteration of the arterial branches of the aortic arch due to arteritis. Acta Psychiatr Neurol Scand 26:313-337
M. Sekiguchi and J. Suzuki: An overview 11. Caccamise WC, Whitman JF (1952) Pulseless disease. Preliminary case report. Am Heart J 44:629-633 12. Ross RS, McKusick VA (1958) Diminished or absent pulses in arteries arising from the arch of the aorta. Aortic arch syndrome. Arch Intern Med 92:701-740 13. Gottsegen G, Szäm I (1956) Über eine eigenartige, unter dem Bilde des brachiozephalischen Arterienverschlusses verlaufende Gefasserkrankung. Z Kreislaufforsch 45:196- 202 14. Koszewski B J, Hubbard TF (1957) Pulseless disease due to brachial arteritis. Circulation 16:406-410 15. Birke G, Ejrup B, Olhagen E (1957) Pulseless disease A clinical analysis of 10 cases. Angiology 8:433-453 16. Correa P, Araujo J (1958) Arteritis of the aorta in young women. Am J Clin Path 29:560-568 17. Lessof MH, Glynn LE (1959) Pulseless syndrome. Lancet 1:799-801 18. Stoyanoff PC (1959) Case of pulseless disease. Takayasu's syndrome. Acta Med Scand 163:167-168 19. Thurlbeck WM, Currens JH (1959) Aortic arch syndrome (pulseless disease). Report of ten cases with three autopsies. Circulation 19:499-510 20. Basu AK (1961) Occlusive disease of the aorta and its main branches. Br J Surg 49:148-156 21. Inada K, Shimizu H, Yokoyama T (1962) Pulseless disease and atypical coarctation of the aorta with special reference to their genesis. Surgery 52:433-443 22. Inada K, Shimizu H, Kobayashi I, Ishiai S, Kawamoto S (1962) Pulseless disease and atypical coarctation of the aorta. Arch Surg 84:306-311 23. Judge RD, Currier RD, Gracie WA, Fingley MM (1962) Takayasu's arteritis and the aortic arch syndrome. Am J Med 32:379-392 24. Hong CY (1963) Pulseless disease with hypertension. Primary arteritis causing occlusion of the left subclavian artery, coarctation of the abdominal aorta and bilateral sclerosis of renal arteries. J Korea Med Assoc 6: 1161-1167 25. Sen PK, Kinare SG, Engineer SD, Parulkar GB (1963) The middle aortic syndrome. Br Heart J 35:610-618 26. Roberts WC, Wibin EA (1966) Idiopathic panaortitis, supra-aortic arteritis, granulomatous myocarditis and pericarditis. Am J Med 41:453-461 27. Nakao K, Ikeda M, Kimata S, Niitani H, Miyahara M, Ishimi Z, Hashiba K, Takeda Y, Ozawa T, Matsushita S, Kuramochi M (1967) Takayasu's arteritis. Clinical report of eighty-four cases and immunological studies of seven cases. Circulation 35:1141-1155 28. Vinijchaikul K (1967) Primary arteritis of the aorta and its main branches (Takayasu's arteriopathy). Am J Med 43:15-27 29. Mufioz N, Correa P (1970) Arteritis of the aorta and its major branches. Am Heart J 80:319-328 30. Reddy GR, Rao NR, Reddy MR et al. (1970) Pathology of pulseless disease. Pathol Microbiol (Basel) 34:10-20 31. Sen PK, Einare SG, Kelkar MD, Parulkar GB (1973) Non-specific aorto-arteritis. A monograph based on a study of 101 cases. McGraw-Hill, Bombay, New Delhi 32. Deutsch V, Wexler L, Deutsch H (1974) Takayasu's arteritis. An angiographic study with remarks on ethnic distribution in Israel. Am J Roentgenol 122:13-28 33. Lupi-Herrera E, Sanchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE (1977) Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 93: 94-103 34. Wiggelinkhuizen J, Cremin BJ (1976) Takayasu arteritis and renovascular hypertension in children. Pediatrics 62:209-217
9 35. Abe K, Miyazaki S (1982) Clinical aspect of aortitis syndrome with special reference to the relation between prognosis and hypertension. Jpn Circ J 46:190-193 36. Fiessinger JN, Tawfik-Taher S, Capron L, Laurian C, Cormier JM, Camilleri JP, Housset E (1982) Maladie de Takayasu. Criteres diagnostiques. Nouv Presse Med 11:583-586 37. Wong VCW, Wand RYC, Tse TF (1983) Pregnancy and Takayasu's arteritis. Am J Med 75:597-601 38. Liu Yu-Qing, Du Jia-Hui (1984) Aorto-arteritis. A collective angiographic experience in 244 cases. Int Angio 3:487-497 39. Numano F, Lee KT, Hong YC (eds) (1992) Takayasu arteritis 1992. Heart Vessels Suppl 7 40. Koide K (1992) Takayasu arteritis in Japan. Heart Vessels Suppl 7:48-54 41. Pokoruny J, Je~kovh Z (1962) Significance of immunological studies in peripheral obliterating vascular diseases. Circ Res 11:961-965 42. Hirch MS, Aikat BK, Basu AK (1964) Takayasu's arteritis. Report of 5 cases with immunologic studies. Bull John Hopkins Hosp 115:29-64 43. Ikeda M (1966) Immunologic studies on Takayasu's arteritis. Jpn Circ J 30:87-89 44. Ito I (1966) Aortitis syndrome with reference to detection of antiaorta antibodies from patients' sera. Jpn Circ J 30:75-78 45. Sano K, Saito I (1966) Immunological atudies of pulseless disease. Neurol Med Chirurg 8:28-39 46. Ueda H, Saito Y, Ito I, Yamaguchi H, Sugiura M, Morooka S (1967) Immunological studies of aortitis syndrome. Jpn Heart J 8:4-18 47. Ito I, Saito Y, Nonaka Y (1975) Immunological aspects of aortitis syndrome. Jpn Circ J 39:458-462 48. Hall S, Nelson AM (1986) Takayasu's arteritis and juvenile rheumatoid arthritis. J Rheumatol 13:431433 49. Scott D, Salmon MI Scott DL, Blann A, Bacon PA, Walton KW, Oakland CD, Slaney GF (1986) Takayasu's arteritis. A pathogenetic role for cytotoxic T lymphocytes. Clin Rheumatol 5:517-522 50. Sakhuja V, Gupta KL, Bhasin DK, Malik N, Chugh KS (1990) Takayasu's arteritis associated with u~cerative colitis. Gut 31:831-833 51. Ask-Upmark E (1954) On the pulseless disease outside of Japan. Acta Med Scand 149:161-178 52. Isaacson C (1961) An idiopathic aortitis in young Africans. J Pathol Bact 81:69-79 53. Lee K, Sohn K, Hong C, Kang S, Berg K (1967) primary arteritis pulseless (pulseless disease) in Korean children. Acta Paediatr Scand 56:526-536 54. Numano F, Isohisa I, Maezawa H, Juji T (1979) HLA antigens in Takayasu disease. Am Heart J 88:153-159 55. Isohisa I, Numano F, Maezawa H, Sasazuki T (1982) Hereditary factors in Takayasu's disease. Angiology 33:98-104 56. Takeda F, Ooneda G, Suto K, Suto H, Kubota K (1961) Pulseless disease. Report of an autopsy case, with special reference to the morphogenesis of the arterial lesions. Gunma J Med Sci 10:27-45 57. Nasu T (1983) Pathology of pulseless disease. A systemic study and clinical review of twenty-one autopsy cases reported in Japan. Angiology 14:225-242 58. Boström K, Hassler O (1965) Takayasu's disease. Postmortem examination of a previously published case. Acta Med Scand 178:537-542 59. Font RL, Naumann G (1969) Ocular histopathology in pulseless disease. Arch Ophthal 82:784-788
10 60. Shionoya S, Griss P (1969) Zur Pathogenese der Takayasu-Krankheit. Arthusphenomen an den Vasa Vasorum. Virchows Arch [A] 348:269-280 61. Nasu T (1976) Takayasu's truncoarteritis in Japan. A statistical observation of 76 autopsy cases. Pathol Microbiol 43:140-146 62. Cipriano PR, Silverman JF, Perloth MG, Gripp RB, Wexler L (1977) Coronary arterial narrowing in Takayasu's arteritis. Am J Cardiol 39:744-750 63. Lande A, Rossi P (1975) The value of total aortography in the diagnosis of Takayasu's arteritis. Radiology 114: 287-297 64. Strachan RW (1964) The natural history of Takayasu's arteriopathy. Q J Med 33:57-69 65. Kinare SG (1970) Aortitis in early life in India and its association with tuberculosis. J Pathol 100:69-76 66. Ishikawa K (1978) Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation 57:27-35 67. Ishikawa K (1981) Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu's disease). Am J Cardiol 47:1026-1032
M. Sekiguchi and J. Suzuki: An overview 68. Ishikawa K, Matsuura S (1982) Occlusive thromboaortopathy (Takayasu's disease) and pregnancy. Clinical course and management of 33 pregnancies and deliveries. Am J Cardiol 50:1293-1300 69. Hayashi K et al. (1986) Takayasu's arteritis. Decrease in aortic wall thickening following steroid therapy, documented by CT. Br J Radiol 59:281-283 70. Ishikawa K, Yonekawa Y (1987) Regression of carotid stenoses after corticosteroidd therapy in occlusive thromboaortopathy (Takayasu's disease). Stroke 18: 677- 679 71. Dong Z et al. (1987) Percutaneous transluminal angioplasty for renovascular hypertension in arteritis. Experience in China. Radiology 162:477-479 72. Seguchi M, Hino Y, Aiba S, Yasukohchi S, Momma K, Takao A, Endo M (1990) Ostial stenosis of the left coronary artery as a sole clinical manifestation of Takayasu's arteritis. A possible cause of unexpected sudden death. Heart Vessels 5:188-191 73. Suzuki A, Amano J, Tanaka H, Sakamoto T, Sunamori M (1989) Surgical consideration of aortitis involving the aortic root. Circulation 80 Suppl 1:222-232
