Takayasu's arteritis: results of a university hospital of 45 patients in Turkey

doi:10.1016/j.ijcard.2003.07.017

International Journal of Cardiology

Volume 96, Issue 2, August 2004, Pages 259-264

https://doi.org/10.1016/j.ijcard.2003.07.017 Get rights and content

Abstract

Background: Takayasu's arteritis (TA) is a rare disease which appears to be most common in East Asia. However, the disease has been reported to be worldwide. The clinical features of the disease can show variations in different geographical areas. The aim of this study is to evaluate clinical, laboratory and radiological features and the outcome of patients with TA in our hospital. Methods: The hospital files of patients who were followed with the diagnosis of TA between the years 1973 and 2003 in Hacettepe University Hospital were retrospectively evaluated. Results: Male/female ratio was 5/40, and the mean age was 34 years (18–59). Constitutional symptoms were present in 71% of the patients. Claudication and pallor of the extremity, decreased extremity pulsations, arterial hypertension, and arterial bruits were present in 44%, 56%, 58%, and 27% of the patients, respectively. Aortic valvular insufficiency, abdominal aortic aneurism, and cardiomegaly were present in four, one, and four patients, respectively. The initial complaint of six patients was cerebrovascular events. The distribution of the patients according to the angiographic findings was as follows, 56% Type I, 18% Type II, 22% Type III, and 4% Type IV arteritis. The need for vascular surgical interventions were significantly less common in patients who were treated with immunosuppressives plus alternate dose steroids (6%) compared to patients who were treated only with antiaggregant agents (33%). Conclusions: The demographic and angiographic findings of our patients were similar to previous observations from Japan and Italy, and disclose distinct clinical features in comparison to other Asian countries. Alternate-day glucocorticoids plus cytotoxic drugs may be beneficial and safe in patients with TA.

Introduction

Takayasu's arteritis (TA) is a rare disease which primarily involves the aorta and its main branches. TA usually affects women of child-bearing age. Female-to-male ratio is 8–10:1 [1]. TA has been reported worldwide but appears to be most common in East Asia [1], [2], [3], [4]. The clinical presentation depends on the location of the affected vessel and the severity of the vascular inflammation. Recent reports from different geographical areas suggested heterogeneity in disease expression in various ethnic populations [2], [3], [4]. Therefore, we aimed to evaluate clinical, laboratory and radiological features and outcome of TA patients in Hacettepe University Hospital in Turkey.

Section snippets

Materials and methods

The hospital files of patients who were followed with the diagnosis of TA between the years 1973 and 2003 in Hacettepe University Hospital were retrospectively evaluated. Demographic, clinical, laboratory and angiographic data were noted. Special attention was given to the therapeutic approaches. The disease activity was determined by using the Burmingham vascular activity score (BVAS) [5].

The diagnoses of TA were reached according to the classification criteria proposed by the American College

Results

Forty-five patients were included in this study. Gender distribution was five males (11%) and 40 females (89%). Mean age was 34 years (min–max: 18–59). Although four patients were above 50 years of age at the time of diagnosis, the symptoms of all those patients had started before 40 years. The mean delay from the onset of the symptoms to the time of diagnosis was 5.01 years (min–max: 3 months–25 years). Constitutional symptoms such as fatigue, malaise, myalgia, arthralgia, palpitations,

Discussion

TA is worldwide disorder of the aorta and its main branches. However, different patterns of clinical, demographic and angiographic features of the disease have been described in different ethnic populations [1], [2], [3], [4]. To our knowledge, only one series of 14 patients has been previously reported from Turkey [9]. In this retrospective and descriptive study, we presented the demographic, clinical and radiological features of 45 patients with TA who were followed in a single referral

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Cited by (40)

Ischemic complications in Takayasu's arteritis: A meta-analysis
2018, Seminars in Arthritis and Rheumatism
Citation Excerpt :
Search results and reasons for exclusion are summarized in Figure 1. The literature search identified 1824 articles; 35 met selection criteria and were included in the analyses (Supplementary Table 2) [4,8–41]. In all, 33 studies reported on stroke and 23 reported on MI.
Takayasu’s arteritis (TAK) is a rare vasculitis affecting the large blood vessels with significant morbidity and mortality from ischemic complications. The objective of this meta-analysis is to determine the proportion of TAK patients with severe ischemic complications.
We performed a literature search using MEDLINE, EMBASE, and the Cochrane library from database inception to March 2016. We included articles that reported at least one severe ischemic complication. A random effects model with inverse-variance weighting was performed to estimate the pooled proportion of TAK subjects with ischemic complications.
In all, 35 studies met inclusion criteria, representing 3262 TAK patients. All studies were observational and of low to moderate quality. Mean age at symptom onset or at diagnosis ranged from 10 to 49 years and mean delay from symptom onset to diagnosis ranged from 2 months to 7.6 years. Study follow-up times were from 22 months to 17 years. The majority of studies had >70% female subjects. The pooled prevalence of stroke in TAK was 8.9% (95% CI: 7.0–10.9%) and of MI was 3.4% (95% CI: 2.1–4.8%) at any time during the disease course. There was moderate-to-severe heterogeneity across the studies (stroke: I² = 64.9%; MI: I² = 74.0%). Other ischemic complications were inconsistently reported.
Stroke and MI are common in TAK patients. Further studies are needed to identify predictors and preventative measures for severe ischemic events in TAK patients.
Incidence, prevalence, mortality and causes of death in Takayasu Arteritis in Korea – A nationwide, population-based study
2017, International Journal of Cardiology
Studies investigating the epidemiology and mortality of Takayasu Arteritis (TA) are scarce. The objective of this study was to provide information regarding the epidemiology of TA, such as the incidence, prevalence, survival and cause of death in Korea.
We used a national, population-based database maintained by the Rare Intractable Disease registration program and the Health Insurance Review and Assessment Service, which includes information on all TA patients, diagnosed based on uniform criteria. We also used data from Statistics Korea linked with RID database to confirm survival and cause of death. We analyzed data for the period 2008–2012.
During study period, there were 612 new patients with TA, with a female-to-male ratio of 4.3:1. The annual mean incidence was 0.24 per 10⁵ people, and the prevalence was 2.82 per 10⁵ people in 2012. The average annual mortality rate was 0.02 per 10⁵ people, and the standardized mortality ratio was 3.1 per 10⁵ people. The overall survival of patients with TA was 96.6%, which was significantly lower than that in the general population. Cardiovascular disease was the most common cause of death, accounting for 29 of the 64 deaths (45.3%), followed by neoplasms in 9 patients (14.1%).
The incidence and prevalence presented in this study are the highest ever reported. The survival of patients with TA was significantly lower than that of the general population, and the main cause of death was cardiovascular disease. This study reflects the need for greater awareness of TA, especially in Asian countries.
An unusual cause of stroke in a young female
2014, American Journal of Emergency Medicine
Citation Excerpt :
Approximately 10% to 20% of patients with TA have been reported to have neurologic manifestations [4,5]. These include dizziness, headaches, and strokes [2,6]. Acute stroke as the first presentation of TA has rarely been reported [7].
Outcome measures in primary systemic vasculitis
2013, Indian Journal of Rheumatology
Citation Excerpt :
However, imaging is likely to play an important part in the future assessment of large vessel vasculitis both in clinical and trial settings. The BVAS has been used in some observational and cohort studies of GCA and TAK.34,35 However, there is a considerable redundancy of items on the BVAS, as it is rare to have pulmonary, renal, ENT, and peripheral neurological involvement with these disorders.
The primary systemic vasculitides are complex multisystem disorder where accurate assessment of disease activity and disease damage is difficult. In order to evaluate therapies in clinical trials good outcome measures are necessary. In ANCA associated vasculitis (AAV), clinical checklists such as the BVAS are used to measure disease activity and the VDI to measure disease and treatment related damage. These tools were developed by consensus expert opinion; have been used for over a decade in clinical trials and are now well-validated and accepted measures. The availability of good outcome measure was a key factor in enabling therapeutic trials in AAV. OMERACT has endorsed a ‘core set’ of domains for trials in AAV with validated measures in each domain. In contrast, there has been an absence of outcome measures in large vessel vasculitis and as a consequence almost no clinical trials in giant cell arteritis or Takayasu's arteritis to date. The landscape may be changing with improved imaging techniques and the recent development of the Indian Takayasu's Arteritis Activity Score (ITAS2010). OMERACT has a working group to try and develop and validate outcome measures for large vessel vasculitis, which provides an opportunity for interested researchers to be involved in this process.
More Than Meets the Eye
2011, Seminars in Arthritis and Rheumatism
A 27-year-old woman was hospitalized with fever and visual changes. She had been well until nine months earlier when she developed unilateral blurry vision in the left eye. Ophthalmologic examination revealed bilateral acute anterior uveitis. She was treated with topical steroids and her vision returned to baseline. However, over the next few months, she developed debilitating fatigue and experienced an unintentional fifteen pound weight loss. One month prior to presentation, she noted the onset of daily low grade fevers and chills. On the day of admission, she developed a temperature of 103°F and a severe frontal headache. Here we describe a case where the overlap of clinical features led to an initially broad differential diagnosis of seemingly unrelated diseases. Ultimately, the discovery of a key radiographic finding allowed us to more clearly define the diagnosis.
Takayasu's Disease in the center of Tunisia: 27 cases
2010, Journal des Maladies Vasculaires
La maladie de Takayasu est une artériopathie inflammatoire rare à propos de laquelle peu de données sont disponibles en Tunisie. Cette étude a pour but de préciser les caractéristiques épidémiologiques, cliniques et radiologiques de cette affection au centre de la Tunisie.
Nous avons rétrospectivement étudié les cas d’artérite de Takayasu suivis dans les services de médecine interne ou de cardiologie des trois centres hospitalo-universitaires de Sousse et Monastir durant la période allant de 1985 à 2005. Les critères d’inclusion choisis étaient ceux de l’American College of Rheumatology.
Vingt-sept patients ont été retenus dont la majorité était des femmes (88,9 % des cas). L’âge moyen au moment du diagnostic était de 33,2 ans (extrêmes 16 et 68 ans) avec un délai diagnostique moyen de 4,2 ans. La claudication artérielle était la principale manifestation révélatrice de la maladie (81,5 % des cas). L’hypertension artérielle était notée chez 40,7 % des patients au moment du diagnostic. La localisation la plus fréquente était représentée par les artères subclavières (96 %). L’aorte était atteinte dans 52,3 % des cas et les artères rénales dans 36,3 % des cas. Les lésions étaient majoritairement sténosantes, les anévrismes n’ayant concerné que deux patientes (7,4 %). Malgré la gêne fonctionnelle gardée par la majorité des patients, la mortalité attribuée à la maladie n’était que de 3,7 % avec un recul moyen de 75,8 mois.
La maladie de Takayasu en Tunisie ne présente pas de différences sur le plan épidémiologique, elle diffère cependant par une fréquence plus élevée de la localisation des troncs supra-aortiques et une fréquence moindre de l’atteinte aortique.
Takayasu's arteritis is a rare inflammatory disease and few data are available in Tunisia. The aim of this study is to evaluate clinical and radiological features of the disease in the centre of Tunisia.
We retrospectively studied medical records of patients treated in departments of internal medicine or cardiology from three university hospitals in Sousse and Monastir over the period 1985–2005. The criteria for inclusion were those proposed by the American College of Rheumatology.
Twenty-seven patients were identified. The mean age at presentation was 33.2 years (range 16–68 years) and 88.9% were female. The mean delay from the onset of the symptoms to the time of diagnosis was 4.2 years. Intermittent claudication was the most common presentation (81.5%) and hypertension was noted in 40.7% of cases. Arterial localization most frequently involved was subclavian artery. The aorta was involved in 52.3% and renal arteries in 36.3% of cases. Stenosis or occlusions was constant but aneurysms were noted in 7.4%.
Functional difficulty was the main complaint in the follow-up, death related to Takayasu's disease was noted in 3.7%. The mean follow-up time was 75.8 months (6.3 years).
There is no epidemiologic particularity of Takayasu's disease in Tunisia, however involvement of the subclavian artery was more frequent than the aortic localization.

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Takayasu's arteritis: results of a university hospital of 45 patients in Turkey

Abstract

Introduction

Section snippets

Materials and methods

Results

Discussion

Lancet

Int. J. Cardiol.

Int. J. Cardiol.

Int. J. Cardiol.

Int. J. Cardiol.

Int. J. Cardiol.

Int. J. Cardiol.

Rheum. Dis. Clin. North Am.

Takayasu's arteritis: a changing disease

J. Nephrol.

Large vessel vasculitides

Curr. Opin. Rheumatol.

Takayasu's arteritis: a case report and discussion of differences in eastern and western cases

Am. J. Cardiovasc. Pathol.

Birmingham vasculitis activity score (BVAS) in systemic necrotizing vasculitis

Q. J. Med.