Takayasu Arteritis: What is the Long-Term Prognosis?

doi:10.1016/j.rdc.2007.07.014

Rheumatic Disease Clinics of North America

Volume 33, Issue 4, November 2007, Pages 777-786

https://doi.org/10.1016/j.rdc.2007.07.014 Get rights and content

Takayasu arteritis (TA) is a form of idiopathic large vessel vasculitis that predominantly affects women of reproductive age. Although TA is a rare disease, the interpretation of longitudinal data from several countries provides new insights into the clinical course and outcomes in TA across different racial and ethnic groups. Contemporary studies belie prior perceptions of TA as a disease with a self-limited, benign course. We now recognize this disease as one that often relapses, leaves the patient chronically dependent on glucocorticoids for disease control, and frequently leads to disability. Limited data suggest that the targeted inhibition of tumor necrosis factor (TNF) might be an effective therapy for TA.

Section snippets

Disease chronicity

Reports from India and Mexico in the era just preceding the availability of noninvasive vascular imaging indicated a high prevalence of patients in a chronic phase, among whom only a minority required ongoing immunosuppressive therapy [5], [6]. This finding led to the perception of TA as a chronic but self-limited disease in most patients.

Earlier studies from Asia, however, had reported morbidity stemming from the progression of vascular lesions and the potential for complications of TA to

Diagnostic guidelines

The age of the population at risk for TA is broader than generally appreciated. The American College of Rheumatology (ACR) classification criteria for TA and Ishikawa's [12], [13] criteria reflect historical perceptions of TA as a disease that predominantly affects young women. In a cohort of 104 patients from Italy who had TA, however, 17% had disease onset after age 40 [14]. Similar findings were observed in two United States cohorts [4], [7], and Asian investigators have reported the

Disease-associated morbidity

The types of morbidity associated with TA are summarized in Fig. 2. We now review the potential for TA to cause morbidity in a variety of organ systems.

Mortality

Lupi-Herrera and colleagues reported a 15% mortality rate among patients who had TA in whom long-term follow-up data were available [17]. Approximately half of the deaths in the cohort resulted from CHF. Among a cohort of 88 patients from India who had TA, there was a 5-year survival rate of 91% and 10-year survival rate of 84%. Forty percent of deaths in the Indian cohort were the result of CHF [18].

CHF was also the most common reported cause of death in a second series from India that

Response to medical therapy

In most cohorts, most patients who have active disease attain disease control with glucocorticoid (GC) therapy. Even among patients who achieve complete remissions on GC therapy, however, relapse is common on GC taper or withdrawal. This phenomenon is reflected in the prevalence of use of other immunosuppressive agents in the Italian (54%), Korean (40%), and American (NIH 42%, CC 73%) cohorts [4], [7], [14], [15]. In the CC longitudinal cohort, 96% of patients who achieved disease remissions

Angioplasty/vascular stents

Initial studies that examined the outcomes of percutaneous transluminal angioplasty (PCTA) in TA seemed promising. In 1992, Sharma and colleagues reported an initial success rate of 82% for renal artery PTCA in the treatment of 33 renal artery lesions in 20 patients who had TA [19]. The patency rate at a mean follow-up of 8 months was 79% [19]. Similar findings were reported by Tyagi and colleagues [20] in a study of 45 patients who had TA, with an initial success rate of 89% and a patency rate

Challenges in disease activity assessment

As in many other disease states, unremitting disease activity can lead to poor outcomes (arterial stenosis and end-organ damage) in TA. A major limitation in the early detection of disease activity of TA is the paucity of reliable clinical symptoms and signs of disease activity at presentation and disease relapse.

Correlation between the levels of acute phase reactants (ie, the erythrocyte sedimentation rate and C-reactive protein) and radiologic findings is often poor in TA. For example, in one

Summary

Longitudinal studies that have included sequential vascular imaging have revealed that in most patients TA is a chronic, relapsing disease that is associated with significant morbidity and disability. The scope of vascular involvement is broader than appreciated two decades ago, before the availability of noninvasive large-vessel imaging studies. High rates of restenosis following vascular interventional procedures, especially during times of overtly active disease, reinforce recommendations to

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