Vol. 116 No. 5 November 2013

Takayasu’s arteritis: oral complications and dental guidelines
Anand Gupta, MDS, MFDS RCPS(Glasgow),a Ongkila Bhutia, MDS,b and
Ajoy Roychoudhury, MDS,c Chandigarh and New Delhi, India
GOVERNMENT MEDICAL COLLEGE AND HOSPITAL AND ALL INDIA INSTITUTE OF MEDICAL SCIENCES

Takayasu’s arteritis (TA) is an inflammatory disease with an unknown cause. It is also known as aortic arch
syndrome and pulseless disease. Complications of TA often include cerebral infarct, valvular heart disease, intracranial
hemorrhage, congestive heart failure, seizures, retinopathy, and renovascular hypertension. We report a case of an 18-yearold female patient with TA who presented with a severe oral infection associated with a mandibular third molar. Such a
complication in patients with TA is rare and this is probably the first reported case in the English medical literature. The
patient was managed satisfactorily, with due precautions and no recurrence. More so, as the prognosis of TA has improved,
the chances of dental treatments of patients afflicted with the same are likely to increase. In addition, this article highlights
the guidelines for the management of patients in various dental specialties. (Oral Surg Oral Med Oral Pathol Oral Radiol
2013;116:e352-e355)

Takayasu’s arteritis (TA) or aortic arch syndrome is an
inflammatory disease with unknown cause. It involves
the aorta, other main blood vessels from the heart, and
also those attached to it. Although it has been reported
worldwide, it is most commonly found in young Asian
women.1 It is also termed as the “pulseless disease”
because pulses on the upper extremities (like radial
pulse), may not be felt.2
Because the prognosis of TA has improved, the
chances of dental treatment are likely to increase in
such patients; however, because of the patients’ debilitated status, oral hygiene may be poor. Consequently,
during the immunosuppressed state, localized infections, such as pericoronitis, may progress into severe
oral infections, such as facial cellulitis. This article
presents a case of a severe oral infection in a rare
disease of TA and guidelines for the management of
the same.

CASE REPORT
An 18-year-old female patient in a wheelchair was brought
to the outpatient department of the Centre for Dental Education and Research, All India Institute of Medical Sciences,
New Delhi, India, by her parents. The chief complaint was
a

Assistant Professor (Oral and Maxillofacial Surgery), Department of
Dentistry, Government Medical College and Hospital (GMCH),
Chandigarh, India.
b
Associate Professor, Department of Oral and Maxillofacial Surgery,
Centre for Dental Education and Research, All India Institute of
Medical Sciences, New Delhi, India.
c
Additional Professor, Department of Oral and Maxillofacial Surgery,
Centre for Dental Education and Research, All India Institute of
Medical Sciences, New Delhi, India.
Received for publication Jul 9, 2011; returned for revision Jan 2,
2012; accepted for publication Jan 18, 2012.
© 2013 Elsevier Inc. All rights reserved.
2212-4403/$ - see front matter
http://dx.doi.org/10.1016/j.oooo.2012.01.036

e352

severe pain and swelling on the right cheek, and pus discharge
from the mouth and face for the preceding 2 weeks. The patient was also finding it difficult to open her mouth. She was
referred from the Department of Rheumatology for the management of facial abscess. She was a known case of TA with
seizure disorder and blurring of vision in both eyes. She was
prescribed antibiotics (amoxicillin 500 mg) and no intervention was made for the management of the facial abscess
before reporting to our department. Further examination revealed that frank pus was draining from both extraoral and
intraoral sinuses (Figure 1, A). The patient’s oral hygiene was
very poor with debris over the right retromolar region. The
pericoronal tissue of the right mandibular third molar was
swollen and erythmatous (Figure 1, B). Severe trismus prevented complete intraoral examination. She was in a serious
condition at the time of presentation, experiencing recurrent
seizures on the dental chair, even during the examination.
Furthermore, it was noted that her radial pulse was absent;
blood pressure could not be recorded in either of her upper
limbs, whereas in the lower limb it was 140/96 mm Hg.
Her medical history revealed a known case of TA for the
preceding 4 years. Concomitantly, she had intermittent claudications and syncopal attacks on the erect posture. She had
a history of hemiparesis with recurrent transient ischemic
attacks. She was undergoing treatment with multiple medical
specialities: rheumatology, interventional cardiology, neurology, and ophthalmology. At the time of presentation at the
maxillofacial outpatient department, the patient was on immunosuppressants (azathioprine 50 mg), corticosteroids
(prednisolone 20 mg), antiepileptics (sodium valproate 50
mg, levetiracetam 100 mg), and antiplatelet (aspirin 150 mg)
drugs.
Funduscopic examination showed ischemic changes in her
retina. Magnetic resonance image of the brain suggested the
presence of an old infarct in the right occipital lobe. The
angiography report showed complete blockage of the left
common carotid artery and bilateral subclavian arteries, an
almost complete blockage of the right common carotid artery,
and thickening of the aortic wall. The activity assessment of
the previous 6 months revealed an active disease with raised

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CASE REPORT
Gupta et al. e353

Fig. 1. A, Preoperative photograph showing swelling over the right cheek region with extraoral sinuses. B, Intraoral photograph
showing trismus and pericoronitis in relation to the right mandibular third molar.

Fig. 2. Orthopantomogram showing bone resorption distal to
the right mandibular third molar and anterior border of ramus.
erythrocyte sedimentation rate, C-reactive protein level, and
arterial wall edema in the computed tomography angiogram.
Blood and radiographic investigations were also performed.
A complete blood count revealed low hemoglobin and leukocytosis with increased polymorphs. Orthopantomogram
showed bone resorption distal to the right mandibular third
molar, also involving a part of the anterior border of ramus
(Figure 2).
On clinical and radiographic examination, the diagnosis of
buccal space infection associated with pericoronitis in relation to the wisdom tooth was made. Initially, the incision and
drainage of pus was done intraorally and injectable broadspectrum antibiotic therapy (co-amoxyclav, a combination of
amoxicillin and clavulanic acid) with analgesics was commenced. Pus culture and sensitivity investigation revealed
Staphylococcus aureus species, sensitive to the combination
of amoxicillin and clavulanic acid. Finally, a similar combination of antibiotics together with local wound care was
maintained for a week. On the third day of the antibiotic
course, approximately 1.5 cm mouth opening was achieved
and the intraoral wound was meticulously debrided and irrigated. After 1 week, the patient’s general condition improved
and pus discharge from both the extraoral and the intraoral
sites was absent. Expert opinion was taken from the rheumatology and neurology departments for a minor surgical procedure under local anesthesia. The procedure was done under
strict cardiac monitoring in the dental chair, in a reclined
position. Under antibiotic coverage and steroid supplementation, extraction of the mandibular third molar and curettage of

bone through the open wound was done under local anesthesia. After 2 weeks, the extraoral sinuses were healed and there
were no signs of infection (Figure 3, A). She regained normal
mouth opening and was encouraged to maintain proper oral
hygiene (Figure 3, B). In the meantime, oral prophylaxis was
done to improve oral health.
During the follow-up visits, pericoronal inflammation
around the opposite side mandibular wisdom tooth was noticed. The offending tooth was extracted prophylactically
under antibiotic coverage to prevent further complications, as
the patient will have to remain on immunosuppressants longterm to control the TA disease. The follow-up examination 2
years later showed no recurrence and the orthopantomogram
showed smooth anterior border of ramus together with complete healing of extraction sockets of the bilateral mandibular
third molars. Adherence to proper oral hygiene measures,
regular dental visits, and checkups were advised.

DISCUSSION
TA was first reported by Takayasu, a Japanese ophthalmologist, in 1908. It was defined as a condition involving a peculiar type of arteriovenous fistulas in the eyes.3
Although its etiology is unknown, some studies suggest
that it may be an autoimmune disorder4 or it may occur
after certain types of infections.1 Although it affects
people of all ethnic backgrounds, this predilection is
most commonly seen in Asian women of childbearing
age.5 The symptoms depend on the intensity and location of the inflammation in the body. Symptoms of TA
may follow a pattern of stages. The first stage may
present with fatigue, malaise, muscle aches, headaches,
joint pain, skin rash, fever, or sometimes weight loss.
The second stage (or vascular inflammatory stage) may
present with pain in the extremities and joints, arm
numbness, blurry or double vision, headaches, or shortness of breath. These symptoms may occur because of
reduced blood supply to the affected area. Occasionally, the symptoms of the first and second stages may
occur together. The third stage is usually associated
with remission, but this stage does not occur in all

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e354 Gupta et al.

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November 2013

Fig. 3. A, Postoperative photograph showing healed extraoral sinuses and healthy skin. B, Intraoral photograph showing adequate
mouth opening and healed intraoral extraction sites-bilateral mandibular third molars.

individuals. Also, the possibility of a relapse must be
noted.6
Laboratory findings may show anemia, raised erythrocyte sedimentation rate, and the C-reactive protein
test may show signs of inflammation.7 Furthermore,
angiography using radiopaque dye can confirm the
presence of the TA disease.
The goal of treatment is to reduce inflammation and
slow or stop the autoimmune disease process. This can
be achieved with corticosteroid medication, such as
prednisone, and it may require administration for a long
time. If corticosteroids do not reduce the symptoms,
cytotoxic drugs, such as methotrexate, azathioprine, or
cyclophosphamide, may be given.6
Recent advances of medical and surgical treatments,
including endovascular interventions, have improved
the prognosis of patients with stenotic/occlusive or
dilated lesions. Several authors have tried to document
predictive factors for the outcome of this disease after
medical treatment.8-10 Among them, the grouping by
Ishikawa8 revealed a good association with the life
expectancy of patients and was adopted by others.9
Miyata et al.11 did a retrospective study to elucidate the
role of surgery in patients with TA by reviewing the
long-term course of 106 consecutive patients treated
surgically. The overall cumulative survival rate at 20
years was found to be 73.5%. The prognostic classification by Ishikawa8 had little influence on the survival
of surgically treated patients. Surgery showed increased
survival for stage 3 patients; however, for stage 1
patients, surgery-related complications conversely decreased survival.11
Complications of TA may include cerebral infarct,
valvular heart disease, intracranial hemorrhage, congestive heart failure, seizures, retinopathy, and renovascular hypertension.12 The reported case is a young Asian

female patient who already had complications of cerebral infarct, seizures, and retinopathy. A thorough
search of the biomedical literature on Medline did not
highlight any case with complications in the maxillofacial region except for one case report on dental specialty that emphasized tooth extraction in Takayasu’s
syndrome.13 The cause of oral infection in the mentioned case could be immunosuppression, owing to
anemia and immunosuppressant drugs. The infection
must have started from the pericoronitis of the wisdom
tooth and spread into the fascial space because of the
patient’s immunocompromised status and poor oral hygiene.
The principles of management of odontogenic infection are to determine the severity of infection, evaluate host defense, treat surgically, support medically,
choose and prescribe antibiotic therapy, administer the
antibiotic properly, and evaluate the patient frequently.
All the mentioned principles were implemented properly and stage-wise treatment was given according to
the compromised status of the patient.14
Yoshimura13 emphasized several problems of tooth
extraction in patients with TA. He also stressed several
points to be taken into consideration during the dental
procedures in such patients. Based on his findings,
some guidelines together with the problem list are
enumerated for the management of patients with TA
during dental procedures.

GUIDELINES FOR DENTAL PROCEDURES IN
PATIENTS WITH TA
During all dental procedures, care must be taken to
position the head in the most relaxed way so there is no
pressure or stretch on the carotid sinus region. Excessive neck bending movements must be avoided because
of impairment or hypersensitivity of the carotid sinus in

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Volume 116, Number 5

some patients with TA, which may lead to bradycardia
or cardiac arrest. All the surgical procedures should be
done in the inactive phase (remission) of the disease.
The activity of this disease can be evaluated from
clinical signs and symptoms (such as easy fatiguability,
general malaise, arthralgia, pain in blood vessels, and
fever) and laboratory investigations, such as erythrocyte sedimentation rate, C-reactive protein level, high
titer of antistreptolysin-O, increased gamma globulin,
and leukocytosis.
Importantly, all dental procedures should be done
under cardiac monitoring. It is beneficial to the operator
to examine the patient’s electrocardiogram, blood pressure, and pulse rate before starting the treatment. Because no pulsation is present at any extremity in many
patients, careful monitoring is essential during treatment. In fact, it is often not realized that the symptoms
derived from hypoperfusion of the cerebral circulation,
which is a result of hyper or impaired reflexes of the
carotid sinus and/or vagal nerve, may be mistaken for a
dental shock. Therefore, it is preferable to treat these
patients in a hospital setting to prevent any unwanted
complications.
Importantly, antibiotic coverage should be given before any surgical procedure. The probable explanation
could be that the bacteremia caused during any dental
surgical procedure may lead to attachment and proliferation of bacteria at the intima of aorta, main branches,
and pulmonary arteries with inflammatory granulation
surfaces. Antibiotic coverage is also important because
of the immunocompromised state of the patients, who
are usually kept on immunosuppressants for the management of this disease.
Most surgical or painful dental procedures should be
done under steroid supplementation, therefore taking
care of any interaction of various prescribed drugs,
such as anticoagulants, antihypertensives, and immunosuppressants. For minor oral and periodontal surgery, evidence suggests that adrenal insufficiency may
be prevented when circulating levels of glucocorticoids
are about 25 mg of hydrocortisone equivalent per day.
This is equivalent to a dosage of about 5 mg of prednisone. In patients taking high dosages of steroid (more
than 5 mg prednisolone), however, the usual dose
should be doubled on the day of the procedure. It is
recommended to schedule the surgery in the morning
hours when normal cortisol levels are at the highest.

CASE REPORT
Gupta et al. e355

In conclusion, dental problems in a patient with TA
are inevitable and their management needs extra precautions and monitoring to avoid any unwanted complications. The enumerated guidelines and the possible
complications may provide enough knowledge of the
disease and give confidence to the dental practitioner to
manage such patients efficiently and satisfactorily.
REFERENCES
1. Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J,
Horwitz S, Vela JE. Takayasu’s arteritis: clinical study of 107
cases. Am Heart J 1977;93:94-103.
2. James WD, Berger TG, Elston DM, Odom RB. Andrews’ diseases of the skin: clinical dermatology. Philadelphia, PA; Saunders Elsevier; 2006. p. 841.
3. Takayasu M. A case with unusual changes of the central vessel
in the retina. Acta Soc Ophth Jap 1908;12:554.
4. Noris M. Physiopathology of Takayasu’s arteritis. J Nephrol
2001;14:506-13.
5. Koening CL, Langford CA. Takayasu’s arteritis. Curr Treat
Options Cardiovasc Med 2008;10:164-72.
6. Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a
review. J Clin Pathol 2002;55:481-6.
7. Choe YH, Han BK, Koh EM, Kim DK, Do YS, Lee WR.
Takayasu’s arteritis: assessment of disease activity with contrastenhanced MR imaging. AJR Am J Roentgenol 2000;175:505-11.
8. Ishikawa K. Patterns of symptoms and prognosis in occlusive
thromboaortopathy (Takayasu’s disease). J Am Coll Cardiol
1986;8:1041-6.
9. Subramanyan R, Joy J, Balakrishnan KG. Natural history of
aortoarteritis (Takayasu’s disease). Circulation 1989;80:429-37.
10. Ishikawa K, Maetani S. Long-term outcome for 120 Japanese
patients with Takayasu’s disease. Clinical and statistical analyses
of related prognostic factors. Circulation 1994;90:1855-60.
11. Miyata T, Sato O, Koyama H, Shigematsu H, Tada Y. Long-term
survival after surgical treatment of patients with Takayasu’s
arteritis. Circulation 2003;108:1474-80.
12. Park MC, Lee SW, Park YB, Chung NS, Lee SK. Clinical
characteristics and outcomes of Takayasu’s arteritis: analysis of
108 patients using standardized criteria for diagnosis, activity
assessment, and angiographic classification. Scand J Rheumatol
2005;34:284-92.
13. Yoshimura Y. Tooth extractions and aortitis syndrome (Takayasu’s disease). Int J Oral Surg 1981;10:1.
14. Miloro M. Peterson’s principles of oral and maxillofacial surgery. 2nd ed. Hamilton (ON): B. C. Decker; 2004. p. 277.
Reprint requests:
Anand Gupta, MDS, MFDS RCPS(Glasgow)
Assistant Professor (Oral and Maxillofacial Surgery)
Department of Dentistry
Government Medical College and Hospital (GMCH)
Sector 32 B
Chandigarh, India Pin code 160030
dranand_kgmc@rediffmail.com