Heart and Vessels

, Volume 7, Supplement 1, pp 11–17 | Cite as

Pathological studies on Takayasu arteritis

  • Masao Hotchi


Takayasu arteritis is a primary inflammatory disease of elastic arteries such as the aorta, its larger branches and the pulmonary artery trunk. According to our recent statistical survey of autopsy cases in Japan, the frequency of the disease in all autopsy cases was approximately 0.033% and the sex ratio was 1:4.5. The most frequent ages of the onset were 20–30 years, those of the death were 40–50 years. The latter was delayed about 20 years in comparison with a previous report. In the recent cases, the vascular lesions widely expanded. Luminal dilatation and aneurysm formation also increased in frequency, their ratio being approximately 57%. In the autopsy cases, the following active lesions were observed: (1) acute exudative inflammation (including suppuration), (2) chronic non-specific productive inflammation and (3) various types of granulomatous inflammation. These findings suggest that many triggers may play a role in the morphogenesis of Takayasu arteritis. The inflammatory lesions are produced in the media and adventitia through the vasa vasorum, and terminate in a diffuse or nodular fibrosis. New active lesions are often observed near the old fibrotic ones. This suggests that Takayasu arteritis may be a progressive disease. Intimal thickening of the peripheral branches from the affected arteries is very often observed. In consequence, secondary ischemic lesions are formed in various organs, especially the heart, brain and kidneys.

Key words

Takayasu arteritis Pulseless disease Aortitis syndrome Elastic arteries 


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  1. 1.
    Takayasu M (1908) A case of peculiar changes in the central retinal vessels (in Japanese). Acta Soc Ophth Jpn 12:554–555Google Scholar
  2. 2.
    Oonishi K (1908) Discussion on Takayasu's presentation (in Japanese). Acta Soc Opth Jpn 12:555Google Scholar
  3. 3.
    Kagoshima S (1908) Discussion on Takayasu's presentation (in Japanese). Acta Soc Opth Jpn 12:555Google Scholar
  4. 4.
    Oota K (1940) Ein seltener Fall von beiderseitigem Carotis-Subclaviaverschluss. Ein Beitrag zur Pathologie der Anastomosis peripapillaris des Auges mit fehlendem Radialpuls. Trans Soc Path Jpn 30:680–690Google Scholar
  5. 5.
    Shimizu K, Sano K (1951) Pulseless disease. J Neuropathol Clin Neurol 1:37–47Google Scholar
  6. 6.
    Nasu T (1963) Pathology of pulseless disease. A systematic study and critical review of twenty-one autopsy cases reported in Japan. Angiology 14:225–242PubMedGoogle Scholar
  7. 7.
    Koide K (1986) Aortitis syndrome. The second statistical survey (in Japanese). Report of the Research Committee on Systemic Vascular Disorders of the Ministry of Health and Welfare, Japan. pp 33–35Google Scholar
  8. 8.
    Nasu T (1975) Takayasu's truncoarteritis in Japan: A statistical observation of 76 autopsy cases. Pathol Microbiol 43:140–146Google Scholar
  9. 9.
    Nagata S (1990) Present state of autopsy cases of Takayasu's arteritis (aortitis syndrome) in Japan (in Japanese). J Jpn Coll Angiol 30:1303–1308Google Scholar
  10. 10.
    Hotchi M (1991) Recent state of autopsy cases of Takayasu arteritis (in Japanese). J Jpn Coll Angiol 31:1213–1216Google Scholar
  11. 11.
    Nasu T (1982) Takayasu's truncoarteritis. Pulseless disease or aortitis syndrome. Acta Pathol Jpn 32 (Suppl 1):117–131PubMedGoogle Scholar
  12. 12.
    Hotchi M (1987) Pathology of aortitis syndrome (in Japanese). Geka 44:691–698Google Scholar
  13. 13.
    Saito K (1988) Pulmonary arteritis in Takayasu's arteritis (in Japanese). Nihon Kyoubu Rinshou 47:655–665Google Scholar
  14. 14.
    Hotchi M, Fujiwara M (1982) Aortitis syndrome (in Japanese). Rinshou Men-eki 14:350–358Google Scholar
  15. 15.
    Hosoda Y, Iri H, Hata J, Wakasugi A (1973) Granulomatous aortitis associated with necrotizing angiitis and glomerulonephritis. Acta Pathol Jpn 23:129–138PubMedGoogle Scholar
  16. 16.
    Takase S, Sato E, Oimomi M, Baba S, Maeda S, Miyazaki K, Sugiyama T (1981) A case of aortitis syndrome in systemic lupus erythematosus (in Japanese). J Jpn Coll Angiol 21:51–54Google Scholar
  17. 17.
    Rush PJ, Inman R, Reynolds WJ (1986) Rheumatoid arthritis after Takayasu's arteritis. J Rheumatol 13:427–430PubMedGoogle Scholar
  18. 18.
    Graham DC, Smythe HA (1958) The carditis and aortitis of ankylosing spondylitis. Bull Rheum Dis 9:171–174PubMedGoogle Scholar
  19. 19.
    Achar KN, Al-Nakib B (1986) Takayasu's arteritis and ulcerative colitis. Am J Gastroenterol 81:1215–1217PubMedGoogle Scholar
  20. 20.
    Yassinger S, Adelman R, Cantor D (1976) Association of inflammatory bowel disease and large vascular lesions. Gastroenterology 71:844–846PubMedGoogle Scholar
  21. 21.
    Zilleruelo GE, Ferrer P, Garcia OL, Moore M, Pardo V, Strauss J (1978) Takayasu's arteritis associated with glomerulonephritis. A case report. Am J Dis Child 132:1009–1013PubMedGoogle Scholar
  22. 22.
    Nagashima M, Nishikawa T (1967) Pyoderma gangrenosum associated with aortitis syndrome (in Japanese). Rinshou Hifuka 21:591–598Google Scholar
  23. 23.
    Yoshii K, Oohata Y, Tashiro M (1978) Pyoderma gangrenosum associated with aortitis syndrome (in Japanese). Nishinihon Hifuka 40:64–71Google Scholar
  24. 24.
    Vinogradova OM, Nevaera EG (1981) Aortic arch syndrome in a patient with primary generalized amyloidosis. Terapenicheskii Arkhiv 53:26–28Google Scholar
  25. 25.
    Walker DI, Bloor K, Williams G, Gillie I (1972) Inflammatory aneurysms of the abdominal aorta. Br J Surg 59:609–614PubMedGoogle Scholar
  26. 26.
    Nakamura T, Ito M, Shiozawa N, Hotchi M (1991) A histopathological study on so-called inflammatory aneurysm of the abdominal aorta. Report of the Research Committee on Intractable Angiitis of the Ministry of Health and Welfare, Japan (in Japanese) pp 31–35Google Scholar

Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • Masao Hotchi
    • 1
  1. 1.Department of PathologyShinshu University School of MedicineMatsumotoJapan

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