Treatment of Takayasu arteritis
- Gene G Hunder, MD
Gene G Hunder, MD
- Section Editor — Vasculitis
- Emeritus Consultant
- Professor Emeritus
- Mayo Clinic College of Medicine
Takayasu arteritis (TA) is a chronic vasculitis of unknown etiology. Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years.
TA primarily affects the aorta and its primary branches (table 1). The initial vascular lesions frequently occur in the left middle or proximal subclavian artery. As the disease progresses, the left common carotid and vertebral, brachiocephalic, right middle or proximal subclavian, right carotid and vertebral arteries, as well as the aorta, may also be affected. The abdominal aorta and pulmonary arteries are involved in approximately 50 percent of patients. (See "Overview of and approach to the vasculitides in adults".)
The inflammatory processes cause thickening of the walls of the affected arteries. The proximal aorta (eg, aortic root) may become dilated secondary to inflammatory injury. Narrowing, occlusion, or dilation of involved portions of the arteries in varying degrees results in a wide variety of symptoms.
The treatment of TA will be reviewed here. The pathogenesis, pathology, clinical manifestations, and diagnosis of this disorder are discussed separately. (See "Clinical features and diagnosis of Takayasu arteritis".)
The mainstay of therapy for Takayasu arteritis (TA) is glucocorticoids. Angioplasty, bypass grafts, or other surgery may be necessary once large aneurysms develop or irreversible arterial stenosis has occurred. Treatment of hypertension and heart failure should be instituted if these complications occur.
- Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am 1995; 21:1041.
- Misra R, Danda D, Rajappa SM, et al. Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010). Rheumatology (Oxford) 2013; 52:1795.
- Hata A, Numano F. Magnetic resonance imaging of vascular changes in Takayasu arteritis. Int J Cardiol 1995; 52:45.
- Sueyoshi E, Sakamoto I, Hayashi K. Aortic aneurysms in patients with Takayasu's arteritis: CT evaluation. AJR Am J Roentgenol 2000; 175:1727.
- Tso E, Flamm SD, White RD, et al. Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. Arthritis Rheum 2002; 46:1634.
- Hoffman GS, Leavitt RY, Kerr GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum 1994; 37:578.
- de Souza AW, da Silva MD, Machado LS, et al. Short-term effect of leflunomide in patients with Takayasu arteritis: an observational study. Scand J Rheumatol 2012; 41:227.
- Daina E, Schieppati A, Remuzzi G. Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases. Ann Intern Med 1999; 130:422.
- Goel R, Danda D, Mathew J, Edwin N. Mycophenolate mofetil in Takayasu's arteritis. Clin Rheumatol 2010; 29:329.
- Salvarani C, Magnani L, Catanoso M, et al. Tocilizumab: a novel therapy for patients with large-vessel vasculitis. Rheumatology (Oxford) 2012; 51:151.
- Unizony S, Arias-Urdaneta L, Miloslavsky E, et al. Tocilizumab for the treatment of large-vessel vasculitis (giant cell arteritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res (Hoboken) 2012; 64:1720.
- Seitz M, Reichenbach S, Bonel HM, et al. Rapid induction of remission in large vessel vasculitis by IL-6 blockade. A case series. Swiss Med Wkly 2011; 141:w13156.
- Salvarani C, Magnani L, Catanoso MG, et al. Rescue treatment with tocilizumab for Takayasu arteritis resistant to TNF-α blockers. Clin Exp Rheumatol 2012; 30:S90.
- Bredemeier M, Rocha CM, Barbosa MV, Pitrez EH. One-year clinical and radiological evolution of a patient with refractory Takayasu's arteritis under treatment with tocilizumab. Clin Exp Rheumatol 2012; 30:S98.
- Valsakumar AK, Valappil UC, Jorapur V, et al. Role of immunosuppressive therapy on clinical, immunological, and angiographic outcome in active Takayasu's arteritis. J Rheumatol 2003; 30:1793.
- Hoffman GS, Merkel PA, Brasington RD, et al. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum 2004; 50:2296.
- Della Rossa A, Tavoni A, Merlini G, et al. Two Takayasu arteritis patients successfully treated with infliximab: a potential disease-modifying agent? Rheumatology (Oxford) 2005; 44:1074.
- Mevorach D, Leibowitz G, Brezis M, Raz E. Induction of remission in a patient with Takayasu's arteritis by low dose pulses of methotrexate. Ann Rheum Dis 1992; 51:904.
- Hall S, Barr W, Lie JT, et al. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore) 1985; 64:89.
- Rao SA, Mandalam KR, Rao VR, et al. Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. Radiology 1993; 189:173.
- 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.
- Liang P, Tan-Ong M, Hoffman GS. Takayasu's arteritis: vascular interventions and outcomes. J Rheumatol 2004; 31:102.
- Park MC, Lee SW, Park YB, et al. Post-interventional immunosuppressive treatment and vascular restenosis in Takayasu's arteritis. Rheumatology (Oxford) 2006; 45:600.
- Matsuura K, Ogino H, Kobayashi J, et al. Surgical treatment of aortic regurgitation due to Takayasu arteritis: long-term morbidity and mortality. Circulation 2005; 112:3707.
- Ishikawa K. Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation 1978; 57:27.
- Eichhorn J, Sima D, Thiele B, et al. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation 1996; 94:2396.
- Schmidt J, Kermani TA, Bacani AK, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clin Proc 2013; 88:822.
- Miyata T, Sato O, Koyama H, et al. Long-term survival after surgical treatment of patients with Takayasu's arteritis. Circulation 2003; 108:1474.
- - Initial glucocorticoid dose
- - Assessing response to treatment
- Clinical findings
- - Tapering glucocorticoids
- - Preventing glucocorticoid adverse effects
- Glucocorticoid-resistant disease
- - Methotrexate
- - Leflunomide
- - Mycophenolate mofetil
- - Tocilizumab
- - Azathioprine
- - Anti-TNF agents
- - Other medications
- - Recommendation
- Aortic valve surgery
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS