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TAKAYASU ARTERITIS - A CASE REPORT:

WITH MAXILLOFACIAL SURGEON‟S

PERSPECTIVE AND BRIEF REVIEW OF

LITERATURE

Dr. Ramesh Srinivasan V, Dr. Priyadharsana PS, Dr. Effie Edsor, Dr.Vigneswaran T, Dr.Gowri Shankar.P*.

Department of Oral and Maxillofacial Surgery

*Department of Oral Pathology

RVS Dental College and Hospital, Coimbatore, Tamilnadu, India.

ABSTRACT

To access & cite this article

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Takayasu arteritis is a chronic inflammatory disease

that affects major vessels, mainly the aorta and its branches.

The diseased condition leads to occlusion, stenosis,

aneurysm or dilatations along the path of the affected artery.

The etiology remains unknown. The disease has been

reported worldwide, with an increased incidence in young

Asian women. Like other medical condition, Takayasu

arteritis patients will seek dental treatment. Hence the

purpose of this article is to highlight the disease features and

what a dentist should know or take precautions before

DOI:10.37841/jidam_2020_v7_i1_05

treating them.

KEYWORDS: Takayasu Arteritis, Pulseless disease,

Autoimmune.

Address for correspondence:

Dr. Effie Edsor, MDS,

Assistant Professor, Department of Oral

and Maxillofacial Surgery,

RVS Dental College and Hospital.

Coimbatore, Tamilnadu, India.

Email id: effieedsor@gmail.com

Received - 28.01.2020

Accepted - 27.02.2020

Published - 27.03.2020

JIDAM/Volume:7/Issue:1/Pages 27 - 31/January - March 2020

Ramesh et al: Takayasu Arteritis in Maxillofacial Surgeons Perspective

INTRODUCTION

million population across the globe.⁶ Based on the recent

epidemiologic study in Europe, the incidence of TA is more

Takayasu arteritis

(TA) is also known as pulseless

with an estimate varying from 0.4 to 1.5 cases per million

disease, aortoarteritis, aortic arch syndrome, idiopathic

population. The highest prevalence of TA is recorded in

aortitis, occlusive thromboaortopathy, occlusive coagulant

Japan (40 cases per million population) and the lowest in US

aortic syndrome, obstructive productive arteritis, reverse

(0.9 per million).⁶ The disease is more common in female

coarction or Martorell syndrome.^1,2,5 TA is a chronic

with the female to male ratio of upto

8:1.² The disease

inflammatory arteritis affecting large vessels, predominantly

commonly presents in 2^nd or 3^rd decade of life but diagnosed

the aorta and its main branches. The most affected branches

in the later stage.⁵

are brachiocephalic, carotid, subclavian, vertebral, renal

arteries, coronary and pulmonary arteries.^3,4 Inflammatory

PATHOPHYSIOLOGY

disease leads to wall thickening, stenosis, fibrosis or

thrombus formation.⁵

The predisposing and etiological factors of TA are still

not clear. An autoimmune basis, influenced by genetic and

HISTORY

environmental factors, is strongly suggested; the resulting

inflammation is largely a cell-mediated immune response.⁷

The disease is named after Mikito Takayasu, an

An association between the extent of vascular involvement

Ophthalmologist, who reported ocular changes like

and the major genetic risk factor HLA-B*52 was found in

aneurysms and arteriovenous anastomoses in patients with

Turkish TA patients, suggesting that genetic factors might

TA (1908).^1,2The disease is named after Takayasu who first

influences the severity of the disease. ^8,9,10

reported a patient with TA, but there are other case reports

of patients with TA in literature reported earlier. In 1830,

CLINICAL FEATURES

Rokushu Yamamoto described a 45-year-old man with TA

with features of persistent fever who later had no pulse in

Clinical manifestations of TA are nonspecific. The most

the right radial artery and weak pulse in the left radial

common clinical features are decreased blood pressure (BP)

artery.² In 1856, Savory presented a case report of TA in

and feeble pulse in the upper extremities, clammy skin and

22-year-old woman, with pulseless disease in both upper

numbness in the fingers.¹¹

extremities and left neck. He later noticed on follow-up that

the patient lost her vision.² However, it was uncertain

The clinical course occurs in two stages; an early active

whether these cases truly suffered from TA.

inflammatory phase and late chronic phase. The duration of

active phase lasts for weeks to months and may have a

Minoru Nakajima in 1921 compared his cases with

remitting and relapsing course. It is characterized by

previous reports and proposed that they should be

systemic symptoms like fever, malaise, night sweats, loss of

considered as one disease. He characterized this disease by

appetite, headaches, dizziness, loss of weight, arthralgia,

the following four criteria:

(i) cases in young women

skin rashes etc. The former phase does not occur in all

affecting bilateral eyes;

(ii) formation of arteriovenous

patients, but indigenous symptoms are often seen in children

anastomosis around the optic disc and microaneurysm in

with TA.¹²

retinal vessels;

(iii) decreased or loss of vision; and

(iv)

unpalpable radial artery. It was Minoru who termed it as

The chronic phase is due to arterial stenosis and/or

„Takayasu disease‟.²Later in 1948, neurosurgeons, Kentaro

occlusion and ischemia of organs.¹³ feeble pulses in 84-96%,

Shimizu and Keiji Sano from the University of Tokyo,

vascular bruits in

80-94%, hypertension in

33-83%,

called the disease as „pulseless disease‟.²

retinopathy upto

37%, aortic regurgitation

20-24%,

congestive cardiac failure, seizures, postural dizziness,

The disease was entitled as „occlusive coagulant aortic

pulmonary artery involvement.⁵ Atypical presentation of

syndrome‟ by Maekawa and Kakei and

„obstructive

homocystinuria in a TA has been recorded in 2013.¹⁴

productive arteritis‟ by Nasu.² In 1965, Riehl et al based on

the pathological and immunological findings, concluded that

DIAGNOSIS

TA is an autoimmune disease.² American College of

Rheumatology published a classification criteria for the

Laboratory test for TA disease tends to be nonspecific.

disease in

1990 and described it as „Takayasu arteritis‟.

The erythrocyte sedimentation rate may be high, generally

Although both „Takayasu arteritis‟ and „Takayasu‟s arteritis‟

greater than

50 mm/h, in early disease but it is often

are used, in the Online Mendelian Inheritance in Man

paradoxically normal later. Leukocyte count may be normal

(OMIM) it is registered as „Takayasu arteritis‟ and this term

or slightly elevated. A moderate, normochromic anaemia

is more commonly used.²

may be present in patients with advanced disease.

Hypoalbuminemia with increased levels of C reactive

INCIDENCE

protein, gamma globulin and fibrinogen are frequent

findings.¹⁵

Takayasu arteritis is a rare disease with worldwide

distribution, being more common in Asian population. The

The American Rheumatological Society 1990 considers

incidence of TA was found to be approximately 1-2 per

three of the following six criteria necessary for a definite

JIDAM/Volume: 7/Issue: 1/Pages 27 - 31/January - March 2020

Ramesh et al: Takayasu Arteritis in Maxillofacial Surgeons Perspective

diagnosis of TA.¹⁶

inhibitors, rituximab and tocilizumab are used in patients

who are resistant to the above mentioned drugs.¹⁹

1) Age of onset before 40 years

2) Claudication of extremities especially upper

The frequency of ischaemic events in TA, may be

extremities

decreased with the use of antiplatelet drugs.¹⁹ A

3) Decreased brachial artery pulse - unilaterally or

retrospective study has concluded that antiplatelet therapy

bilaterally

was associated with decreased frequency of ischaemic

4) BP difference > 10 mm Hg in systolic blood

events in patients with TA.^22,23

pressure between both arms

5) On auscultation bruit over subclavian arteries or

Surgical management involves balloon angioplasty or

abdominal aorta

stent graft replacement. In long standing cases, surgical

6) Arteriogram abnormality

- narrowing or

bypass of the affected segment is required.¹⁹As a general

occlusion

rule, surgical intervention should be avoided during the

active phase of the disease.¹⁹ Earlier diagnosis, better

ISHIKAWA’S CRITERIA FOR THE DIAGNOSIS OF

assessment of disease activity and clinical trials will improve

TAKAYASU’S ARTERITIS ¹⁷

the prognosis of TA.

Obligation criterion

CASE REPORT

Age < 40 year (Symptoms of more than 1month

duration)

A female patient aged

45 years reported to the

outpatient ward with a chief complaint of shock like pain on

Two major criteria.

left side of the face for past 8 years. Pain was of sudden

onset, severe intensity with very short duration, radiating to

Stenosis or occlusion in the

the left shoulder. The pain aggravated in the morning and

1) Left mid subclavian artery

relieved in the noon. Patient was treated for trigeminal

2) Right mid subclavian artery.

neuralgia for 4 years (gabapentin plus and zeptol)

Nine minor criteria.

Patient had the history of headache and before 4 years,

she noticed numbness in both the upper limbs, percutaneous

1) Raised ESR (persistent high ESR >20 mm/h)

retrogasserian glycerol rhizotomy (PRGR) attempted but failed

2) Carotid artery tenderness (Unilateral or bilateral)

due to no cerebrospinal fluid (CSF) flow. She complained the

3) Persistent high blood pressure

140/90mmHg

history of weight loss of around 8 kg in past 5 years. With

brachial or >160/90mmHg popliteal

previous history and CT angiographic findings (Fig 1), patient

4) Aortic regurgitation

was diagnosed as TA. Patient noticed watering of the left eye

5) Pulmonary artery lesion (Lobar or segmental arterial

before 5 months and was advised steroid therapy. On intra-oral

occlusion)

examination, patient had poor oral hygiene and grossly decayed

6) Lesion in left mid common carotid artery

36. Left radial artery couldn‟t be palpated; the pulse and blood

7) Distal brachiocephalic trunk lesion

pressure were under normal limit. Since the patient was known

8) Descending thoracic aorta lesion

case of TA, with physician consent, under antibiotic coverage,

9) Abdominal aorta lesion

extraction of

36 was done due to poor prognosis. Patient

reported for review with no fresh complaint, hence oral

In addition to obligatory criterion, the presence of major

prophylaxis was done. The patient was advised to maintain oral

criteria or of one major and two or more minor criteria or of

hygiene and have a periodic dental visit. Since the patient was

four minor criteria suggests high probability of the disease.¹⁷

not on antiplatelet or on long term steroid, procedure was done

Conventional radiographic angiography [digital subtraction

only on antibiotic cover.

angiography

(DSA)] is the gold standard technique for

diagnosis of TA.¹⁸Recently non-invasive imaging methods

including magnetic resonance angiography (MRA), colour

doppler ultrasound

(CDU), computerized tomography

angiography

(CTA), PET with

18F-fluorodeoxyglucose

(18F-FDG) and 18F-FDG PET/CT have recently gained

ground on DSA.^19,20,21

MANAGEMENT

Corticosteroids and conventional immunosuppressive

agents such as methotrexate (MTX), azathioprine (AZA),

mycophenolic acid (MMF) and leflunomide (LEF) are the

Fig 1: CT Angiogram of patient

most commonly used agents for TA management.¹⁹

Biological drugs such as

(TNF) tumour necrosing factor

MAXILLOFACIAL SURGEON’S PERSPECTIVE

JIDAM/Volume: 7/Issue: 1/Pages 27 - 31/January - March 2020

Ramesh et al: Takayasu Arteritis in Maxillofacial Surgeons Perspective

Onen F, Akkoc N. Epidemiology of Takayasu arteritis.

Adequate knowledge and proper diagnosis play a

La presse medicale. July-August 2017; 46(7-8): e197-

key role in the management of TA patients since it is a rare

e203.

disease. Like other medical condition, early morning

appointments to be made to avoid stress. During all dental

Marson A, Housley WJ, Hafler DA. Genetic basis of

procedures, patients head should be positioned in a relaxed

autoimmunity. The Journal of Clinical Investigation.

way to avoid pressure or stress on the carotid sinus region

June 2015;125(6): 2234-41.

which might lead to bradycardia.¹² Gupta et al in his article

has mentioned that dental surgical procedures preferably to

Saruhan-Direskeneli G, Hughes T, Aksu K,Keser G,

be performed in the inactive phase

(remission) of the

Coit P, Aydin S et al. Identification of multiple genetic

disease.¹²

susceptibility loci in Takayasu arteritis. Am J Hum

Genet. 2013;93(2):298-30.

Extraction or other surgical procedures should be

carried out under antibiotic cover to prevent bacteremia and

Sahin Z, Bicakcigil M, Aksu K, Kamali S, Akar S,

proliferation in the inflamed vessels. Most of TA patients are

Onen F et al. Takayasu‟s arteritis is associated with

on high dose steroid therapy during the active phase. In such

HLA-B*52, but not with HLA-B*51, in Turkey.

situation, the dosage of steroid must be doubled (rule of two)

Arthritis Res Ther 2012;14(1): R27.

to prevent adrenal crisis.

10.

Renauer P, Sawalha AH. The genetics of Takayasu

The TA patients are prescribed antiplatelet therapy to

arteritis. Presse Med. 2017, 46, e179-e187.

prevent ischemic complications. Hence before surgical

procedures cardiovascular monitoring should be done.

11.

Mohan S, Lakshmi R. A case report on Takayasu‟s

Arteritis. Int J Pharm Pharm Sci. 2017; 9(3): 296-297.

CONCLUSION

12.

Sadurska E, Jawniak R, Majewski M, Czekajska-

Maxillofacial and dental problems are inevitable in

Chehab E. Takayasu arteritis as a cause of arterial

any patient. Detailed history, knowledge about the disease

hypertension. Case report and literature review. Eur J

and management of medically compromised patient with

Pediatr. 2012;171(5):863-869.

extra precautions is mandatory to avoid complications.

13.

Khan MAM, Banoo H. A case report of Takayasu‟s

FINANCIAL SUPPORT AND

arteritis. Medicine today. 2012; 24(02):79-81.

SPONSORSHIP:

14.

Manocha D, Kumar K, Anand R. Atypical presentation

Nil

of a young female patient with Takayasu Arteritis: Case

report and review of literature. Int J Clin Pediatr.

CONFLICTS OF INTEREST:

2013;2(1):34-36.

There are no conflicts of interest.

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Manfrini O, Bugiardini R. Takayasu‟s arteritis: A case

report and a brief review of the literature. Heart

International 2006;2(1):66-71.

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