Takayasu’s arteritis: a cell-mediated large-vessel vasculitis

  • R. Rizzi
  • S. Bruno
  • C. Stellacci
  • R. Dammacco


Takayasu’s arteritis is an idiopathic, systemic inflammatory disease, typically involving the aorta and its main branches. Cell-mediated autoimmunity has been strongly implicated in its pathogenesis. Early or active-stage pathology consists of continuous or patchy granulomatous inflammation, which progresses to intimal and adventitial fibrosis and scarring of the media. Multiple focal or segmental stenoses result and aneurysms may occasionally occur. Clinical presentation is heterogeneous, ranging from asymptomatic to catastrophic. In some patients, constitutional signs and symptoms indicating a systemic inflammatory response are observed, usually in the early stages. Specific features reflect arterial involvement, and result from end-organ or limb ischemia; they include vascular, neurological, cardiac, and pulmonary manifestations. The course of Takayasu’s arteritis usually extends for many years with varying degrees of activity. Takayasu’s arteritis has a worldwide distribution, with the greatest prevalence in eastern countries. Women of reproductive age are preferentially affected, but the illness is being recognized with increasing frequency in males. Variable phenotypes are recently emerging in different ethnic groups. Diagnosis is based on clinical features and vascular imaging studies that document typical patterns of stenoses or aneurysms of the aorta and its primary branches. Assessment of the activity of Takayasu’s arteritis is imprecise, in that clinical features and acute-phase reactants do not accurately reflect active blood vessel inflammation. High-dose corticosteroids alone or a cytotoxic agent in addition to a corticosteroid may be effective in treating active disease. Critical lesions may require correction by surgery or interventional radiology.

Key words

Takayasu’s arteritis Large-vessel vasculitis T cells Arteriographic types 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Seko Y, Minota S, Kawasaki A, Shinkai Y, Maeda K, Yagita H, Okumura K, Sato O, Takagi A, Tada Y, Yazaki Y. Perforin-secreting killer cell infiltration and expression of a 65-kD heat-shock protein in aortic tissue of patients with Takayasu’s arteritis. J Clin Invest 1994; 93: 750.PubMedCrossRefGoogle Scholar
  2. 2.
    Kerr G. Takayasu’s arteritis. Curr Opin Rheumatol 1994; 6: 32.PubMedCrossRefGoogle Scholar
  3. 3.
    Nityanand S, Giscombe R, Srivastava S, Hjelmström P, Sanjeevi CB, Sinha N. A bias in theαβT cell receptor variable region gene usage in Takayasu’s arteritis. Clin Exp Immunol 1997; 107: 261.PubMedCrossRefGoogle Scholar
  4. 4.
    Lie J and members and consultants of the American College of Rheumatology Subcommittee on classification of vasculitis: illustrated histopathologic classification criteria for selected vasculitis syndromes. Arthritis Rheum 1990; 33: 1074.PubMedGoogle Scholar
  5. 5.
    Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW Jr, Masi AT, McShane DJ, Mills JA, Stevens MB, Wallace SL, Zvaifler NJ. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33: 1129.PubMedCrossRefGoogle Scholar
  6. 6.
    Hoffman GS. Treatment of resistant Takayasu’s arteritis. Rheum Dis Clin North Am 1995; 21: 73.PubMedGoogle Scholar
  7. 7.
    Hunder G. Vasculitis: diagnosis and therapy. Am J Med 1996; 100 [Suppl 2A]: 37S.Google Scholar
  8. 8.
    Morales E, Pineda C, Martínez-Lavín M. Takayasu’s arteritis in children. J Rheumatol 1991; 18: 1081.PubMedGoogle Scholar
  9. 9.
    Martini A. Behçet’s disease and Takayasu’s disease in children. Curr Opin Rheumatol 1995; 7: 449.PubMedCrossRefGoogle Scholar
  10. 10.
    Di Giacomo V, Meloni F, Transi MG, Nigro D, Sciacca V. Takayasu’s disease in middle aged women. A clinicopathologic study. Angiology 1985; 36: 70.PubMedCrossRefGoogle Scholar
  11. 11.
    Sabbadini MG, Bozzolo E, Baldissera E, Angeli E, Bianchi R, Rugarli C. Storia naturale e follow-up terapeutico della malattia di Takayasu (abstract). Abstracts of the 99th National Congress of the Society of Internal Medicine. Bari, 10–14 November, 1998; Abstract book: 106.Google Scholar
  12. 12.
    Cid MC, Font C, Coll-Vinent B, Grau JM. Large vessel vasculitis. Curr Opin Rheumatol 1998; 10: 18.PubMedCrossRefGoogle Scholar
  13. 13.
    Weyand CM, Goronzy JJ. Molecular approaches toward pathologic mechanisms in giant cell arteritis and Takayasu’s arteritis. Curr Opin Rheumatol 1995; 7: 30.PubMedCrossRefGoogle Scholar
  14. 14.
    Numano F. Differences in clinical presentation and outcome in different countries for Takayasu’s arteritis. Curr Opin Rheumatol 1997; 9: 12.PubMedCrossRefGoogle Scholar
  15. 15.
    Savory WS. Case of a young woman in whom the main arteries of both upper extremities and of left side of the neck were throughout completely obliterated. Med Chir Trans Lond 1856; 39: 205.Google Scholar
  16. 16.
    Takayasu M. Case with unusual changes of the central vessels in the retina (in Japanese). Acta Soc Ophthalmol Jpn 1908; 12: 554.Google Scholar
  17. 17.
    Shimizu K, Sano K. Pulseless disease. J Neuropathol Clin Neurol 1951; 1: 37.Google Scholar
  18. 18.
    Mehra NK, Rajalingam R, Sagar S, Jain S, Sharma BK. Direct role of HLA B5 in influencing susceptibility to Takayasu aortoarteritis. Int J Cardiol 1996; 54 [Suppl]: S71.Google Scholar
  19. 19.
    Kimura A, Kitamura H, Date Y, Numano F. Comprehensive analysis of HLA genes in Takayasu arteritis in Japan. Int J Cardiol 1996; 54 [Suppl]: S61.Google Scholar
  20. 20.
    Seko Y, Sato O, Takagi A, Tada Y, Matsuo H, Yagita H, Okumura K, Yazaki Y. Restricted usage of T-cell receptor Vα-Vβ genes in infiltrating cells in aortic tissue of patients with Takayasu’s arteritis. Circulation 1996; 93: 1788.PubMedGoogle Scholar
  21. 21.
    Sagar S, Ganguly NK, Koicha M, Sharma BK. Immunopathogenesis of Takayasu arteritis. Heart Vessels 1992; 7 [Suppl]: 85.CrossRefGoogle Scholar
  22. 22.
    Sharma BK, Jain S, Ganguly NK. Intracellular signal transduction in T cells in Takayasu’s arteritis. Ann NY Acad Sci 1996; 793: 453.PubMedCrossRefGoogle Scholar
  23. 23.
    Scott DG, Salmon M, Scott DL, Blann A, Bacon PA, Walton KW, Oakland CDH, Slaney GF. Takayasu’s arteritis: a pathogenic role for cytotoxic T lymphocytes. Clin Rheumatol 1986; 5: 517.PubMedGoogle Scholar
  24. 24.
    Blann AD. Takayasu’s arteritis (letter). Intern Med 1995; 237: 217.Google Scholar
  25. 25.
    Imiela J, Gorski A, Dybowska B, Malecki R, Nosarzewski J. Disturbances of T-cell interactions with endothelium and the extracellular matrix in a patient with Takayasu arteritis. J Intern Med 1994; 235: 609.PubMedGoogle Scholar
  26. 26.
    Hellmann DB. Immunopathogenesis, diagnosis, and treatment of giant cell arteritis, temporal arteritis, polymyalgia rheumatica, and Takayasu’s arteritis. Curr Opin Rheumatol 1993; 5: 25.PubMedCrossRefGoogle Scholar
  27. 27.
    Eichhorn J, Sima D, Thiele B, Lindschau C, Turowski A, Schmidt H, Scheneider W, Haller H, Luft FC. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation 1996; 94: 2396.PubMedGoogle Scholar
  28. 28.
    Rizzi R, Liuzzi GM, Frassanito MA, Bruno S, Cafforio P. Antiendothelial cell antibodies (AECA) in a patient with Takayasu’s arteritis (abstract). Abstracts of the Second Symposium on Pathogenesis and Immunotherapy of Autoimmune Diseases. Milan, 25–27 October 1996; Milan: Roche Milano Ricerche: 115.Google Scholar
  29. 29.
    Nityanand S, Mishra K, Shrivastava S, Holm G, Lefvert AK. Autoantibodies against cardiolipin and endothelial cells in Takayasu’s arteritis: prevalence and isotype distribution. Br J Rheumatol 1997; 8: 923.CrossRefGoogle Scholar
  30. 30.
    Rizzi R, Stellacci C, Bruno S, Dammacco R. Anti-endothelial cell autoantibodies in Takayasu’s arteritis (abstract). Reumatismo 1998; 50 [Suppl 3]: 405.Google Scholar
  31. 31.
    Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG. Takayasu arteritis. Medicine (Baltimore) 1985; 64: 89.Google Scholar
  32. 32.
    Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, Hoffman GS. Takayasu arteritis. Ann Intern Med 1994; 120: 919.PubMedGoogle Scholar
  33. 33.
    Hoffman GS. Takayasu’s arteritis: lessons from American National Institute of Health experience. Int J Cardiol 1996; 54S: 99.CrossRefGoogle Scholar
  34. 34.
    Dammacco F. Arterite di Takayasu (sindrome dell’aorco aortico o malattia senza polso). In: Teodori U, Gentilini P, eds. Trattato di medicina interna. Rome: SEU; 1993; 867–870.Google Scholar
  35. 35.
    Condorelli M, Marone G, Triggiani M. Arterite di Takayasu. In: Dammacco F, ed. Immunologia in medicina. Milan: Ermes; 1989: 917–921.Google Scholar
  36. 36.
    Dammacco F, Perosa F, Rizzi R, Sansonno D, Silvestris F, Vacca A. Vasculiti primarie sistemiche. Atti of the 99th National Congress of the Society of Internal Medicine. Bari, 10–14 November 1998. Roma: Ed. Pozzi: 1–116.Google Scholar
  37. 37.
    Michel BA, Arend WP, Hunder GG. Clinical differentiation between giant cell (temporal) arteritis and Takayasu’s arteritis. J Rheumatol 1996; 23: 106.PubMedGoogle Scholar
  38. 38.
    Chugh KS, Sakhuja V. Takayasu’s arteritis as a cause of renovascular hypertension in Asian countries. Am J Nephrol 1992; 12: 1.PubMedCrossRefGoogle Scholar
  39. 39.
    Yamato M, Lecky WL, Hiramatsu K, Kohda E. Takayasu arteritis: radiographic and angiographic findings in 59 patients. Radiology 1986; 161: 329.PubMedGoogle Scholar
  40. 40.
    Vanoli M, Miani S, Amft N, Bacchiani G, Radelli L, Scorza R. Takayasu’s arteritis in Italian patients. Clin Exp Rheumatol 1995; 13: 45.PubMedGoogle Scholar
  41. 41.
    Biswas A, Banerjee AK, Roy S, Khan AK, Haldar B, Roychowdhuri GK, Chatterjee SS, Maity AK. Eye changes in non-specific aorto-arteritis. J Indian Med Assoc 1996; 94: 17.PubMedGoogle Scholar
  42. 42.
    Ishikawa K, Maetani S. Long-term outcome for 120 Japanese patients with Takayasu’s disease. Clinical and statistical analyses of related prognostic factors. Circulation 1994; 90: 1855.PubMedGoogle Scholar
  43. 43.
    Ishikawa K. Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu’s disease). Am J Cardiol 1981; 47: 1026.PubMedCrossRefGoogle Scholar
  44. 44.
    Subramanyan R, Joy J, Balakrishnan KG. Natural history of aortoarteritis (Takayasu’s disease). Circulation 1989; 80: 429.PubMedGoogle Scholar
  45. 45.
    Sabbà C, Pugliese D, Caruso G, Ettorre G, Bianco M, Centonze V, Pennella A, Albano O. Takayasu’s arteritis: a case report and discussion of differences in eastern and western cases. Am J Cardiovasc Pathol 1990; 3: 96.Google Scholar
  46. 46.
    Sharma BK, Jain S, Suri S, Numano F. Diagnostic criteria for Takayasu arteritis. Int J Cardiol 1996; 54 [Suppl]: S141.Google Scholar
  47. 47.
    Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol 1997; 54 [Suppl]: S155.Google Scholar
  48. 48.
    Girona E, Amigo MC, Izaguirre R, Banales JL, Reyes PA. Takayasu arteritis. IV. Absence of autoantibodies and coagulation/fibrinolysis abnormalities. Rev Invest Clin 1993; 45: 241.PubMedGoogle Scholar
  49. 49.
    Akazawa H, Ikeda U, Kuroda T, Shimada K. Plasma endothelin-1 levels in Takayasu’s arteritis. Cardiology 1996; 87: 303.PubMedCrossRefGoogle Scholar
  50. 50.
    Boehme MW, Schmitt WH, Youinou P, Stremmel WR, Gross WL. Clinical relevance of elevated serum thrombomodulin and soluble E-selectin in patients with Wegener’s granulomatosis and other systemic vasculitides. Am J Med 1996; 101: 387.PubMedCrossRefGoogle Scholar
  51. 51.
    Matsunaga N, Hayashi K, Sakamoto I, Ogawa Y, Matsumoto T. Takayasu arteritis: protean radiologic manifestations and diagnosis. Radiographics 1997; 17: 579.PubMedGoogle Scholar
  52. 52.
    Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan — new classification of angiographic findings. Angiology 1997; 48: 369.PubMedCrossRefGoogle Scholar
  53. 53.
    Chung JW, Park JH, Im JG, Chung MJ, Han MC, Ahn H. Spiral CT angiography of the thoracic aorta. Radiographics 1996; 16: 811.PubMedGoogle Scholar
  54. 54.
    Park JH. Conventional and CT angiographic diagnosis of Takayasu arteritis. Int J Cardiol 1997; 54 [Suppl]: S165.Google Scholar
  55. 55.
    Suwanwela N, Piyachon C. Takayasu arteritis in Thailand: clinical and imaging features. Int J Cardiol 1996; 54 [Suppl]: S117.Google Scholar
  56. 56.
    Rainen RO, Kupari MM, Pamilo MS, Pajari RI, Poutanen VP, Hekali PE. Arterial wall thickness measurements by B mode ultrasonography in patients with Takayasu’s arteritis. Ann Rheum Dis 1996; 55: 461.CrossRefGoogle Scholar
  57. 57.
    Hoffmann GS, Leavitt RY, Kerr GS, Rottem M, Sneller MC, Fauci AS. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum 1994; 37: 578.CrossRefGoogle Scholar
  58. 58.
    Langford CA, Sneller MC, Hoffman GS. Methotrexate use in systemic vasculitis. Rheum Dis Clin North Am 1997; 23: 841.PubMedCrossRefGoogle Scholar
  59. 59.
    Cantini F, Niccoli L, Padula A, Olivieri I. Therapy of Takayasu arteritis with cyclosporin A (abstract). International Congress on Immunointervention. Rome, Italy, 20–22 June 1996. Abstract book: 131.Google Scholar
  60. 60.
    Regina G, Fullone M, Testine M, Todisco C, Greco L, Rizzi R, Caruso G, Ettorre GC. Aneurysms of the supra-aortic trunks in Takayasu’s disease. Report of two cases. J Cardiovasc Surg 1998; 39: 1.Google Scholar
  61. 61.
    Beale PG, Meyers KEC, Thomson PD. Management of renal hypertension in children with Takayasu’s arteritis using renal autografting or allograft transplantation in selected circumstances and total lymphoid irradiation. J Pediatr Surg 1992; 27: 836.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1999

Authors and Affiliations

  • R. Rizzi
    • 1
  • S. Bruno
    • 1
  • C. Stellacci
    • 1
  • R. Dammacco
    • 2
  1. 1.Department of Biomedical Sciences and Human Oncology, Section of Internal Medicine and Clinical OncologyUniversity of Bari Medical SchoolBariItaly
  2. 2.Department of Ophthalmology and Otorhinolaryngology, Section of OphthalmologyUniversity of Bari Medical SchoolBariItaly

Personalised recommendations