Diagnostic Features, Treatment, and Outcomes of Takayasu Arteritis in a US Cohort of 126 Patients

doi:10.1016/j.mayocp.2013.04.025

Mayo Clinic Proceedings

Volume 88, Issue 8, August 2013, Pages 822-830

https://doi.org/10.1016/j.mayocp.2013.04.025 Get rights and content

Abstract

Objective

To describe the clinical features, treatment, and outcomes in a longitudinal cohort of patients with Takayasu arteritis (TAK).

Patients and Methods

We retrospectively studied patients with newly diagnosed TAK evaluated from January 1, 1984, through December 31, 2009.

Results

The cohort included 126 patients who were predominantly white (85/103; 82.5%) and female (115/126; 91%). The median age at diagnosis was 31.6 years (interquartile range, 22.9-39.8 years). Median delay in diagnosis was 17.5 months (interquartile range, 7-41.8 months). Thirty-one patients (25%) were 40 years or older at diagnosis. Median delay in diagnosis for patients 40 years or older was 44.8 months compared with 28.3 months for those younger than 40 years (P<.001). Limb claudication was the presenting symptom in 64 of 123 patients (52%). Hata type V arteriographic abnormalities were the most common (57/100; 57%). Renal artery abnormalities were observed in 24 of 41 patients (58%) with new-onset hypertension. Inflammatory markers were elevated at diagnosis in 85 of 119 patients (71%). Vascular interventions were performed in 69 patients (55%). Seventy-nine patients (63%) were followed up for more than 1 year (median follow-up, 5.5 years; interquartile range, 2.9-10.0 years). In this subset, treatment consisted of corticosteroids in 73 patients (92%) and additional immunosuppressants in 52 patients (66%). At 5 years, 96% experienced at least one remission of any duration. The overall survival was 97% at 10 years and 86% at 15 years. Mortality was increased compared with the general population (standardized mortality ratio, 3.0; 95% CI, 1.0-8.9).

Conclusion

There continues to be an unacceptably long delay in the diagnosis of TAK. Awareness of TAK in patients older than 40 years is needed. Morbidity was high despite immunosuppressive treatment. Survival was decreased in this cohort.

Section snippets

Patients and Methods

In this retrospective cohort study, all patients evaluated at the Mayo Clinic in Rochester, Minnesota, from January 1, 1984, through December 31, 2009, with an International Classification of Diseases, Ninth Revision code for TAK were identified. All medical records were reviewed to confirm the diagnosis. We included patients who met the American College of Rheumatology (ACR) criteria for TAK.⁵ Patients 41 to 50 years of age who otherwise met the TAK ACR criteria without fulfilling ACR criteria

Study Cohort

During the study period, 126 patients (91% female; n=115) were diagnosed as having TAK at Mayo Clinic or were evaluated at our institution within 1 year of the diagnosis. The ACR criteria for TAK modified for age were fulfilled in 117 patients (93%). An additional 9 patients (7%) who did not meet modified ACR criteria for TAK but were deemed to have TAK by the study team were also included in this cohort. The demographic characteristics of the cohort are summarized in Table 1.

Clinical Findings at Diagnosis

Symptoms,

Discussion

In this article, we describe the clinical features, treatment, and outcomes in a large cohort of patients with TAK seen at a single tertiary referral center in North America. There is a long delay between symptom onset and diagnosis in patients with TAK. This was especially true in patients older than 41 years. Although remission was achieved in most patients, relapses were common. More than half of the cohort of patients underwent surgical or interventional procedures. Morbidity was high

Conclusion

There continues to be an unacceptable delay in the diagnosis of patients with TAK. Despite similar clinical manifestations at diagnosis, patients 40 years or older had a markedly longer delay in diagnosis than those younger than 40 years. Despite treatment, relapses are common and disease-associated morbidity remains high. Although survival in this cohort was similar to what has been reported in other cohorts of patients with TAK, we observed increased mortality compared with that in the

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The clinical characteristics of Malaysian patients with Takayasu Arteritis: A retrospective study of 3 decades
2024, Egyptian Rheumatologist
To understand the clinical features, angiographic involvement patterns and outcomes of Takayasu Arteritis (TA) in Malaysian patients.
This was a retrospective study of 85 TA patients from 20 tertiary rheumatology centers in Malaysia from 1990 until 2020. Demographic information, clinical features, angiographic patterns, treatment and comorbidities were analyzed.
The female-to-male ratio was 13.1:1. The age at disease onset was 27.47 ± 10.4 years. The duration of delay in diagnosis was 6 ± 27.5 months. 48.2 % were Malay, 12.9 % Chinese, 23.5 % Indian and 14.3 % other ethnicities. Hypertension emerged as the most common comorbidity (52.9 %), followed by dyslipidemia (29.4 %). The most prevalent angiographic pattern was Type V (55.3 %), with left subclavian artery (65.9 %) and left carotid (55.3 %) being the most commonly involved vessels. Glucocorticoids constituted the mainstay of treatment; however, approximately half of the patients required treatment with methotrexate and azathioprine. There were six deaths, primarily attributed to coronary artery disease and infections. Surgical interventions, including angioplasty and bypass surgery, were performed on 11 patients. Out of 44 pregnancies, 79.5 % were successful.
This study revealed a pattern of TA disease in Malaysia that aligns with findings from other cohort studies. The most prevalent angiographic type observed was type V, with the subclavian and carotid arteries being the most commonly involved vessels among TA patients in Malaysia. This research contributes to a better understanding of the clinical presentations observed over the past three decades, offering valuable insights for the improved management of TA patients in Malaysia.
Neurological manifestations of Takayashu's disease
2023, Pratique Neurologique - FMC
La maladie de Takayashu est une vascularite systémique rare de cause inconnue, qui intéresse essentiellement les femmes jeunes. Ubiquitaire, elle est plus fréquemment rencontrée en Asie. Les atteintes artérielles concernent essentiellement l’aorte et ses branches principales, ainsi que les grosses artères à type de parois épaissies, de sténoses, d’occlusions, de dilatations ou d’anévrysmes, à l’origine de signes cliniques ischémiques variés. Parmi elles, les manifestations neurologiques sont fréquentes, parfois révélatrices, et constituent un facteur de mauvais pronostic. Il n’existe à ce jour aucun biomarqueur fiable pour le diagnostic. Néanmoins, la caractérisation des lésions vasculaires est rendue plus facile aujourd’hui grâce aux nouvelles techniques d’imagerie non invasives telles que l’angiographie par résonance magnétique et la tomographie par émission de position couplée à la tomodensitométrie marquée au fluorodéoxyglucose. Cet article a pour objectif de détailler ces manifestations à la lumière des progrès d’imagerie récents, pour un diagnostic et une prise en charge thérapeutique plus précoces. Il en abordera également les principaux aspects épidémiologiques, cliniques, diagnostiques et thérapeutiques.
Takayashu's disease is a rare systemic vasculitis of unknown etiology. It is more common in young women and has a worldwide distribution, although it is more frequently seen in Asia. Arterial damage mostly involves the aorta and its major branches, as well as large arteries, including thickening of the vessel wall, stenosis, occlusion, dilation, and aneurysms, leading to various clinical signs of ischemia. Among them, neurological manifestations, considered as a prognostic factor, are frequent and sometimes inaugural. Nowadays, there is no reliable biomarker for the diagnosis of Takayashu's disease, but new noninvasive imaging modalities such as magnetic resonance angiography (MRA) and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) make it easier to characterize vascular lesions of the disease. This article aims to detail these manifestations in the light of the latest advances in imaging, which allow earlier diagnosis and treatment. It also addresses the principal epidemiological, clinical, diagnostic, and therapeutic aspects of Takayashu's disease.
Predictors of relapse in Takayasu arteritis
2023, European Journal of Internal Medicine
Takayasu arteritis (TAK) is a large-vessel vasculitis with high relapse rate. Longitudinal studies identifying risk factors of relapse are limited. We aimed to analyze the associated factors and develop a risk prediction model for relapse.
We analyzed the associated factors for relapse in a prospective cohort of 549 TAK patients from the Chinese Registry of Systemic Vasculitis cohort between June 2014 and December 2021 using univariate and multivariate Cox regression analyses. We also developed a prediction model for relapse, and stratified patients into low-, medium-, and high-risk groups. Discrimination and calibration were measured using C-index and calibration plots.
At a median follow-up of 44 (IQR 26–62) months, 276 (50.3%) patients experienced relapses. History of relapse (HR 2.78 [2.14–3.60]), disease duration <24 months (HR 1.78 [1.37–2.32]), history of cerebrovascular events (HR 1.55 [1.12–2.16]), aneurysm (HR 1.49 [1.10–2.04], ascending aorta or aortic arch involvement (HR 1.37 [1.05–1.79]), elevated high-sensitivity C-reactive protein level (HR 1.34 [1.03–1.73]), elevated white blood cell count (HR 1.32 [1.03–1.69]), and the number of involved arteries ≥6 (HR 1.31 [1.00–1.72]) at baseline independently increased the risk of relapse and were included in the prediction model. The C-index of the prediction model was 0.70 (95% CI 0.67–0.74). Predictions correlated with observed outcomes on the calibration plots. Compared to the low-risk group, both medium and high-risk groups had a significantly higher relapse risk.
Disease relapse is common in TAK patients. This prediction model may help to identify high-risk patients for relapse and assist clinical decision-making.
Takayasu Arteritis: JACC Focus Seminar 3/4
2023, Journal of the American College of Cardiology
Takayasu arteritis is a rare idiopathic large-vessel vasculitis that typically affects young women. An early "prepulseless" stage is often missed, associated with nonspecific constitutional symptoms (fever, malaise, and weight loss) and elevated inflammatory markers. Unchecked disease progression leads to the "pulseless" stage, manifest clinically by missing pulses, vascular tenderness, and ischemic symptoms (limb claudication, dizziness, angina, and renovascular hypertension), and is characterized pathologically by arterial wall thickening and stenotic/occlusive lesions or aneurysm formation. Vascular complications (stroke, blindness, heart failure, and aneurysm rupture) could follow unless disease progression is halted by immunosuppressive therapy and critical lesions are palliated by timely endovascular therapy or open surgery. Early diagnosis, effective therapy, and lifelong surveillance for disease activity relapses and vascular disease progression are critical to successful long-term outcomes. The outlook for patients has improved significantly in recent years with the establishment of diagnostic and classification criteria, better investigational modalities, and more effective medical and invasive therapy.
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Citation Excerpt :
High C-reactive protein levels and aortic arch involvement are more commonly reported in treatment-resistant TAK. Vascular involvement tends to be progressive, but survival rates >90% are reported at 5 years of follow-up.54 Longer term, the presence of complications (aortic regurgitation, aneurysm, hypertension) and disease progression are major predictors of outcomes at 15 years with survival rates of 68% and 93% for those with and without a progressive course, respectively.
Inflammatory aortitis is most often caused by large vessel vasculitis (LVV), including giant cell arteritis, Takayasu’s arteritis, immunoglobulin G4–related aortitis, and isolated aortitis. There are distinct differences in the clinical presentation, imaging findings, and natural history of LVV that are important for the cardiovascular provider to know. If possible, histopathologic specimens should be obtained to aide in accurate diagnosis and management of LVV. In most cases, corticosteroids are utilized in the acute phase, with the addition of steroid-sparing agents to achieve disease remission while sparing corticosteroid toxic effects. Endovascular and surgical procedures have been described with success but should be delayed until disease control is achieved whenever possible. Long-term management should include regular follow-up with rheumatology and surveillance imaging for sequelae of LVV.

View all citing articles on Scopus

: Grant Support: This study was supported by the Mayo Foundation.

: Potential Competing Interests: Dr Schmidt is the recipient of a scholarship from the Conseil Régional de Picardie. Dr Kermani was supported by the Vasculitis Clinical Research Consortium, which has received support from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54AR057319), the National Center for Research Resources (U54 RR019497), and the Office of Rare Diseases Research. The Vasculitis Clinical Research Consortium is part of the Rare Diseases Clinical Research Network.

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Original articleDiagnostic Features, Treatment, and Outcomes of Takayasu Arteritis in a US Cohort of 126 Patients

Abstract

Objective

Patients and Methods

Results

Conclusion

Section snippets

Patients and Methods

Study Cohort

Clinical Findings at Diagnosis

Discussion

Conclusion

Am Heart J

Int J Cardiol

Int J Cardiol

J Vasc Surg

Medium- and large-vessel vasculitis

N Engl J Med

Advances in the medical and surgical treatment of Takayasu arteritis

Curr Opin Rheumatol

Takayasu arteritis: a study of 32 North American patients

Medicine (Baltimore)

Takayasu arteritis

Ann Intern Med

The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis

Arthritis Rheum

The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis

Arthritis Rheum

Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients

Arthritis Rheum

Takayasu's arteritis: a study of 104 Italian patients

Arthritis Rheum

Improved prognosis of Takayasu arteritis over the past decade—comprehensive analysis of 106 patients

Circ J

Development of outcome measures for large-vessel vasculitis for use in clinical trials: opportunities, challenges, and research agenda

J Rheumatol

Original article
Diagnostic Features, Treatment, and Outcomes of Takayasu Arteritis in a US Cohort of 126 Patients