ORIGINAL ARTICLE

Retrospective evaluation of 22 patients with Takayasu’s arteritis

Nazife Sule Yasar Bilge • Timucin Kasifoglu •

Dondu U. Cansu • Cengiz Korkmaz

Received: 20 August 2010 / Accepted: 30 December 2010 / Published online: 20 January 2011

� Springer-Verlag 2011

Abstract Takayasu’s arteritis (TA) is a rare, idiopathic,

inflammatory, granulomatous vasculitis that affects the

aorta and its primary branches. Clinical features and the

pattern of arterial involvement show differences in differ-

ent regions of the world according to ethnic influences. Our

aim in this retrospective study was to evaluate the demo-

graphic, clinic, laboratory, and angiographic findings of 22

patients with TA followed by our clinic and also compare

our results with series from the literature. The hospital files

of the 22 patients followed by our clinic between 1998 and

2009 were retrospectively evaluated. We also compared

our results with the series from the literature that we were

able to reach by US National Library of Medicine, National

Institute of Health. Gender distribution, age at diagnosis,

and type of aortic involvement were similar with the study

from Turkey. Different clinical manifestations of Taka-

yasu’s arteritis have been described in different ethnic

groups. We also want to underline the coincidence of TA

and other rheumatic diseases such as sarcoidosis, SLE, RA,

and psoriatic arthritis, different from other published series.

Keywords Takayasu’s arteritis � Vasculitis � Pulseless

disease � Immunosuppressive treatment

Introduction

Takayasu’s arteritis (TA) is an uncommon vasculitis that

primarily affects the aorta and its main branches [1, 2].

Inflammation may involve entire vessel wall causing ste-

notic or occlusive lesions in 98% of patients and aneurisms

in 27% [1]. Even TA is most commonly seen in East Asia,

the disease has been reported worldwide [2]. The incidence

ranges between 1.2 and 2.6 cases/million/year [3]. TA

occurs more frequently in women than in men; female to

male ratio is 8–10:1 [4]. Clinical manifestations are non-

specific such as myalgias, arthralgias, fever, weight loss,

carotidynia, and symptoms reflecting stenosis of the

effected arteries [5]. The diagnoses can delay until patients

have symptoms such as transient ischemic attack or stroke,

results in the localized stenosis of the affected artery [1, 2].

Vascular imaging is absolutely necessary for the correct

diagnosis and also to monitorize the disease progression

[2]. Clinical features and arterial involvement patterns

show differences in different regions of the world [6]. The

aim of this retrospective study was to evaluate the demo-

graphic, clinic, laboratory, and angiographic findings of 22

patients with TA followed by Eskisehir Osmangazi Uni-

versity, Faculty of Medicine, Division of Rheumatology.

We have also compared our results with the series from

other countries to present the differences and similarities

between populations.

Methods

The hospital files of the 22 patients who fulfilled the

American College of Rheumatology 1990 criteria for the

diagnosis of TA and followed by our clinic between 1998

and 2009 were retrospectively evaluated. Data included

patients’ gender, age, age of disease, symptoms at admis-

sion, and laboratory findings including erythrocyte sedi-

mentation rate (ESR), C-reactive protein (CRP), and

therapy (medical or surgical). The pretreatment and

N. S. Y. Bilge (&) � T. Kasifoglu � D. U. Cansu � C. Korkmaz

Faculty of Medicine, Internal Medicine,

Division of Rheumatology, Eskisehir Osmangazi University,

Eskisehir, Turkey

e-mail: suleyasar@yahoo.com

123

Rheumatol Int (2012) 32:1155–1159

DOI 10.1007/s00296-010-1764-z

posttreatment ESR and CRP values were compared with

Wilcoxon signed-rank test. Patients underwent aortic

angiography at the time of diagnosis and were classified

into 6 types using the new classification of Takayasu’s

arteritis according to the international conference on

Takayasu’s arteritis, 1994 [6]. Type I involves branches of

aortic arch; Type IIa involves ascending aorta, aortic arch,

and its branches; Type IIb is a combination of Type IIa plus

involvement of thoracic descending aorta; Type III

involves thoracic descending aorta, abdominal aorta, and/

or renal arteries; Type IV involves only abdominal aorta

and/or renal arteries; Type V is a combination of Type IIb

plus Type IV.

We have also compared our results with the series from

the literature that we were able to reach by US National

Library of Medicine, National Institute of Health, and

written in English.

Results

Demographic and laboratory features

Twenty-two patients were included in the study. Nineteen

of the 22 patients (86.3%) were woman. Mean age of the

patients was 39.6 ± 12.1, age at onset of disease was

30.1 ± 12.3 (ranged between 12 and 60), and average age

at diagnosis was 30.1 ± 12.3. The mean follow-up period

was 4.7 ± 3.2 years changing from 1 month to 10 years

(Table 1).

The mean erythrocyte sedimentation rate (ESR) was

55.6 ± 35.5 mL/h (0–20 mL/h) and mean C-reactive pro-

tein (CRP) was 4.6 ± 4.9 mg/dL (0–0.5 mg/dL) before

treatment and 31.1 ± 23.4 mL/h and 1.9 ± 3.4 mg/dL

after treatment, respectively (Table 1).

Both acute-phase reactants decreased after treatment.

The decrease was statistically significant between pre-

treatment and posttreatment ESR (P = 0.008), but the

difference was insignificant between pretreatment and

posttreatment CRP (P = 0.3).

Comorbid diseases

Some patients had sarcoidosis, systemic lupus erythema-

tosus (SLE), rheumatoid arthritis (RA), and psoriatic

arthritis in medical history. Of the patients, 22.7% had

hypertension and 4.5% of them had diabetes mellitus at the

time of diagnosis. One of the patients had bilateral uveitis

at the time of diagnosis.

Clinical features

Clinical features of the patients were nonspecific. Claudi-

cation of the extremity was present in 10 patients (45.5%),

and 18.2% of the patients had vertigo. Carotidynia and

fatigue were present in 13.6% of the patients. Visual

symptoms, arthritis, fever, abdominal pain, and diarrhea

were present in 9.1% of the patients. Headache, nausea,

syncope, and raynaud phenomenon were present in 4.5% of

the patients. Loss of hearing was the initial complaint of

one patient (Table 2).

In physical examination, 90.9% of the patients had

murmur in cervical region, 54.5% had murmur in abdom-

inal region, and 33.3% had in both regions.

Type of aortic involvement

Seventeen of the 22 patients had diagnosis by X-ray

angiography, two by MR angiography, two by CT angi-

ography, and one of them was defined with thorax CT.

The most common type of aortic involvement was Type

I arteritis (36.4%); 22.7% had Type IV involvement; and

18.2% had Type V arteritis. Type II aortic involvement was

the least common arteritis (9.1%), and 45.5% of the

Table 1 Demographic and laboratory features of the patients with

TA

F/M 19/3

Mean age (years) 39.6 ± 12.1

Age at diagnosis 30.1 ± 12.3

Mean follow-up period (years) 4.7 ± 3.2 (1–120 months)

ESR before treatment 55.6 ± 35.5 mL/h

ESR after treatment 30.1 ± 23.4 mL/h

CRP before treatment 4.6 ± 4.9 mg/dL

CRP after treatment 1.9 ± 3.4 mg/dL

Table 2 Clinical features of the patients with TA

Clinical features Number

of patients

%

Claudication 10 45.5

Vertigo 4 18.2

Carotidynia 3 13.6

Fatigue 3 13.6

Visual symptoms 2 9.1

Arteritis 2 9.1

Fever 2 9.1

Abdominal pain 2 9.1

Diarrhea 2 9.1

Headache 1 4.5

Nausea 1 4.5

Syncope 1 4.5

Raynaud phenomenon 1 4.5

Loss of hearing 1 4.5

1156 Rheumatol Int (2012) 32:1155–1159

123

patients had renal arterial involvement. Unilateral and

bilateral involvement was equal (18.2%), and 20% of the

patients had aortic aneurism. Three patient’s angiographic

data were missing (Table 3).

Aortic aneurism was seen in three patients. Aneurism

developed in one patient with HT and retinopathy devel-

oped in another.

Treatment

Therapeutic approaches to TA patients included both

medical treatment and surgical treatment; 76.2% received

steroids, 52.4% had methotrexate, and 47.6% had aza-

thioprine therapy. The number of patients who had lef-

lunamide or infliximab was 2 (9.5%). The average

therapy period was 3.6 years (1–10 years). Both patients

had infliximab therapy in combination with azathioprine.

One of the patients received methotrexate in addition to

azathioprine. Infliximab was used as an alternative

treatment in patients who were evaluated as resistant to

azathioprine and methotrexate therapies. Surgery was

performed to 7 patients (33.3%); 22.7% had bypass

surgery and stent placement was performed to 9.1% of

them (Table 4). None of the patients had undergone a

second surgical procedure.

Outcome

One of our patients died in coronary intensive care unit

because of congestive cardiomyopathy. And four of our

patients were lost to follow up.

Discussion

TA has an unpredictable pattern with various clinical

manifestations [2]. Different manifestations of the disease

have been described in different ethnic groups [3]. There

are two series with 45 and 14 patients have been reported

from Turkey previously [3, 7]. In this retrospective study,

we presented demographic, clinical, laboratory, and

angiographic findings of 22 patients with TA followed by

our clinic and compared our results with the other series

published before.

The mean age and clinical features of our patients were

similar with the published series from Serbia, Colombia,

India, North America, Korea, and also from our country

[2, 4, 8–13]. In India, the female:male ratio was 1.58:1 and

2.15:1 in Thailand [9, 13, 14] (Table 5). In many of the

published studies including ours, the female predominance

is more significant. In a study reported by Lupi-Herrera

et al. [10], the onset of age was less than 20, much more

younger than the other series. Diagnosis was delayed

almost 2 years after the beginning of the symptoms like

mentioned in other studies [11]. This is because of the

nonspecificity of the symptoms. The patients can be diag-

nosed earlier if the clinician is suspicious about TA in

differential diagnosis.

The most common symptom was claudication (45.5%)

same as the patients from Colombia and North America

[8, 11]. In the study by Ureten et al. [4], claudication was

the second common manifestation (44%) following con-

stitutional symptoms (71%).

Of the patients, 90.9% had murmur in the neck, same as

those in the literature [8]. Even this is an important indi-

cator of the disease, it is not diagnostic. Hypertension was

found in a less percentage of patients different from the

series of Colombia, India, North America, and Thailand

[8–11, 14], but HT is a multifactorial originated disease

that can explain the difference.

Type I aortic involvement, involving branches of

aortic arch, was the most common type among the

patients. Same involvement was seen among the patients

from Serbia, Colombia, and Korea, but Type V

involvement was more common in series from Japan,

India, and Thailand [8, 9, 12, 14]. Our results were also

similar to the other studies from Turkey [4]. Renal

arterial involvement was more common among the

Indian patients [9].

Table 3 Type of aortic involvement

Type Number of patients %

Type I 8 36.4

Type IIa 0 0

Type IIb 2 9.1

Type III 0 0

Type IV 5 22.7

Type V 4 18.2

Involvement of renal arteries

(unilateral/bilateral)

8 (4/4) 36.4 (18.2/18.2)

Table 4 Therapeutic approaches to the patients

Treatment Number of

the patients

%

Bypass surgery 5 22.7

Stent 2 9.1

Steroid 16 72.7

Methotrexate 11 50

Azathioprine 10 45.5

Leflunomide 2 9.1

Infliximab 2 9.1

Rheumatol Int (2012) 32:1155–1159 1157

123

We have defined active disease using clinical signs,

symptoms, and increase in acute-phase reactants (ESR and

CRP). Resolution of clinical and laboratory findings were

defined as remission. Using immunosuppressive treatment

provides remission.

Glucocorticoids are the effective palliative agents for

most of the patients. But the addition of the immunosup-

pressive agents provides remission in higher rates [5, 6],

and in our experience, several patients needed immuno-

suppressive therapy to control the disease activity. The

most commonly used immunosuppressive drug was meth-

otrexate same as the published studies [6]. In the published

study from Serbia [2], 69% of the patients needed a second

immunosuppressive agent other from glucocorticoids. We

have treated all patients with combination therapy includ-

ing glucocorticoids and other immunosuppressive agents

same as Ureten et al. [4]. Infliximab was used as an

alternative treatment in resistant patients. There are some

studies in the literature supporting the usage of anti-tumor

necrosis factor therapy in Takayasu’s arteritis and resulted

in remission [15, 16]. Infliximab might be an effective

alternative treatment choice in Takayasu’s arteritis.

Also, in the same study from Serbia, 25% of the patients

required multiple surgical interventions. None of our

patients had undergone a second surgical intervention.

But restenosis was reported in 24.23% of the cases in India

[13].

In conclusion, some of the demographic and clinical

findings of our patients were similar to those reported

before and some of them were different from the published

series. The difference might be related to ethnic influences.

The similarities between our data and Ureten et al.’s results

support this consequence. In addition, we want to underline

the coincidence of TA and other rheumatic diseases. Five

of our patients had TA secondary to a rheumatic disease

such as SLE, RA, sarcoidosis, and psoriatic arthritis. There

are some TA cases that were presented as sarcoidosis, SLE,

and RA [17, 18], but ours had sarcoidosis, SLE, RA, and

psoriatic arthritis and developed TA later on progress.

These uncommon associations of the two such rare dis-

eases in the same person raise questions of common etio-

logic factors.

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5C

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par

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fro

mli

tera

ture

Co

un

try

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Co

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Ind

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No

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Am

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Pre

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Nu

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nts

73

35

10

63

21

29

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16

45

22

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(13

–4

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–4

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52

)3

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8–

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)3

0.1

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icin

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ent

I(2

1%

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(34

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(6.6

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(11

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%)

III

(4%

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(27

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V(1

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%)

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