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ESSENTIALS OF DIAGNOSIS

  • Takayasu arteritis (TAK) causes granulomatous vasculitis of the aorta, main aortic branches and pulmonary arteries.

  • Preferentially affects young women.

  • Often presents with absent pulses, extremity claudication, hypertension, or constitutional symptoms.

  • Erythrocyte sedimentation rate and C-reactive protein levels are usually elevated.

  • Definitive diagnosis requires vascular imaging or biopsy.

  • Patients respond acutely to prednisone. However, relapse upon prednisone tapering is common and glucocorticoid-sparing immunosuppressive drugs are needed for remission maintenance in most cases

  • Subclinical progression of arterial damage despite treatment can be seen and patients should undergo periodic imaging surveillance.

Takayasu arteritis (TAK), named for the Japanese ophthalmologist who first described the ocular manifestations in 1908, is a granulomatous large-vessel vasculitis that mostly affects women during their reproductive years. The granulomatous arteritis associated with TAK leads both vascular stenoses and ectases, depending on the specifc vessel involved. TAK generally presents two major challenges. First, the diagnosis can be delayed for months or even years due to its rarity and the protean nature of the presenting manifestations. Second, relapse and subclinical disease progression often occur despite treatment. Although TAK is a chronic condition, it usually follows a waxing and waning course that requires careful clinical and imaging monitoring to determine when the disease is active, and immunosuppression is needed. Because of the advances in medical therapy and surgical treatment of vascular complications such as aortic regurgitation, survival of patients with TAK has increased significantly. Substantial morbidity, however, still occurs in most cases. Morbidity occurs not only as a result of the disease but also from complications of treatment.

Epidemiology

TAK has been reported most extensively in Asia, the Middle East, and Latin America, but cases have been described worldwide and TAK is known to affect patients with diverse ethnic backgrounds (Onen and Akkoc, 2017). The highest prevalence of TAK, 40 cases per million population, was estimated in Japan. The prevalence in the United States has been reported to be 0.9–2.6 cases per million population. The prevalence in European countries varies between 4.7 and 33 per million population. TAK affects women up to eight times more frequently than men (Goel et al, 2018). The average age at diagnosis is in the mid-20s, but the disease may begin as early as age 4 or as late as age 74. Symptoms develop before age 20 in nearly one-third of patients and after age 40 in 10–25% of them. Caucasians tend to be older at the time of diagnosis.

Etiology & Pathogenesis

The cause of TAK remains elusive. However, the geographic clustering of cases suggests genetic or environmental etiologic factors. HLA and non-HLA genetic associations have been identified. These include HLA-Bw52, HLA-B/MICA, HLA-DQB1, HLA-DRB1, FCGR2A, FCGR3A, RPS9/LILRB3, IL6, TNF-α, IL-17F, and IL12B (Carmona et al, 2017; Renauer et al, 2015; Saruhan-Direskeneli et al, 2013). In addition, the predominance of TAK in women of childbearing age suggests that ...

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