Giant Cell Arteritis 515

• Headache, often associated with marked
BASIC INFORMATION
DEFINITION
Giant cell arteritis (GCA) is a segmental systemic
granulomatous arteritis affecting medium and
large arteries in individuals >50 yr. Inflammation
primarily targets branches of the extracranial
head and neck blood vessels (external carotids,
temporal arteries, ciliary and ophthalmic arteries).
The aorta and subclavian and brachial arteries
can also be affected. Intracranial arteritis is rare.
for diagnosing GCA is described in Fig. 2. The
scalp tenderness—noticed while brushing
hair (hair comb allodynia).
• Constitutional symptoms (fever, weight loss,
anorexia, fatigue).
• Polymyalgia rheumatica (aching and stiffness
of the trunk and proximal muscle groups).
• Visual disturbances (transient or permanent
monocular or binocular visual loss).
• Intermittent claudication of jaw and tongue
on mastication that is especially prominent
when solid food such as steak is chewed.
• Table 1 describes atypical manifestations of
American College of Rheumatology has pro-
posed classification criteria to aid in the diagno-
G
sis of GCA. Presence of three or more of these
criteria in a patient with suspected vasculitis is
considered to be suggestive of GCA.
• Age of onset of symptoms >50 yr.
• New-onset of or new type of localized
headache.
• Temporal artery abnormalities including ten-
derness or decreased pulsation.
• Westergren erythrocyte sedimentation rate
(ESR) elevated (typically >50 mm/hr).

and Disorders
Diseases
SYNONYMS GCA. • Temporal artery biopsy with vasculitis and
Temporal arteritis Important physical findings in GCA: mononuclear cell infiltrate or granulomatous
Cranial arteritis • Vascular examination: The temporal artery dem- changes.
GCA onstrates tenderness, decreased pulsation, and
nodularity (ropy) (Fig. 1); diminished or absent DIFFERENTIAL DIAGNOSIS
Horton’s disease
ICD-10CM CODES
pulses in upper extremities may be seen. • Other vasculitic syndromes.
• Nonarteritic anterior ischemic optic neuropa- I
M31.5 Giant cell arteritis with polymyalgia ETIOLOGY thy (NAION).
rheumatica Vasculitis of unknown etiology. Recent demon- • Pituitary apoplexy.
M31.6 Other giant cell arteritis stration of varicella zoster virus virion, antigen, • Primary amyloidosis.
and DNA within the vessel walls of the temporal • Transient ischemic attack, stroke.
arteries on histopathologic specimens of giant • Infections.
EPIDEMIOLOGY & cell arteritis suggest an association. • Occult neoplasm, multiple myeloma.
DEMOGRAPHICS
INCIDENCE: Approximately 20 new cases per LABORATORY TESTS
100,000 persons >50 yr; peak incidence is in DIAGNOSIS
• 
ESR elevated although up to 22% of
patients ages 60 to 80 yr. Clinical history and vascular examination patients with GCA have normal ESR before
PREVALENCE: 200 cases per 100,000 persons; remain cornerstones of diagnosis. An algorithm treatment.
it is the most common primary vasculitis;
female/male predominance of twofold to four-
fold; more common in Caucasians.

PHYSICAL FINDINGS & CLINICAL

PRESENTATION
GCA can present with the following clinical
manifestations:

TABLE 1  Atypical Manifestations

of Giant Cell Arteritis

Fever of unknown origin

Respiratory symptoms (especially cough)
Otolaryngeal manifestations
Glossitis A B
Lingual infarction
Throat pain
Hearing loss
Large-artery disease
Aortic aneurysm
Aortic dissection
Limb claudication
Raynaud’s phenomenon
Neurologic manifestations
Peripheral neuropathy
Transient ischemic attack (TIA) or stroke
Dementia
Delirium
Myocardial infarction
Tumorlike lesions
Breast mass
Ovarian and uterine mass
C D
Syndrome of inappropriate antidiuretic hormone FIG. 1  Giant cell arteritis.  A, Histology shows transmural granulomatous inflammation, disruption of the
secretion (SIADH) internal elastic lamina, proliferation of the intima, and gross narrowing of the lumen. B, The superficial temporal
Microangiopathic hemolytic anemia
artery is pulseless, nodular, and thickened. C, Ischemic optic neuropathy. D, Ischemic optic neuropathy and
From Harris ED et al: Kelly’s textbook of rheumatology, ed 7, cilioretinal artery occlusion. (From Kanski JJ, Bowling B: Clinical ophthalmology, a systematic approach, ed 7,
Philadelphia, 2005, Saunders. Philadelphia, 2010, Saunders.)

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516 Giant Cell Arteritis
Giant cell arteritis suspected
Temporal artery biopsy
Positive biopsy: Negative biopsy
GCA proven
GCA still
strongly suspected
Perform second biopsy of
temporal artery or occipital
artery or perform imaging
study if large artery
involvement suspected
FIG. 2  Algorithm for diagnosing giant cell arteritis (GCA). (From Firestein GS et al: Kelly’s textbook of
rheumatology, ed 9, Philadelphia, 2013, Saunders.)
• C-reactive protein (CRP) is typically included
in laboratory investigation; it may have great-
er sensitivity than ESR. CRP typically rises
before the ESR.
• Mild to moderate normochromic normocytic
anemia, elevated platelet count.
IMAGING STUDIES
• Color duplex ultrasonography (CDUS) of
temporal artery produces three character-
istic features—periluminal “halo” over the
temporal artery involved, segmental arterial
stenosis, and arterial luminal occlusion in
severe cases. CDUS of the temporal artery
has 40% to 75% sensitivity and 79% to 83%
specificity for diagnosis of GCA. Clinical
utility is not superior to clinical examination
with biopsy.
• Contrasted MRI of temporal artery may be
performed in patients with contraindications
to surgical biopsy of the superficial temporal
artery, if treatment with steroids has not
been initiated. MRI has a 78.4% sensitivity
and 90.4% specificity in detecting temporal
artery involvement in patients with a clinical
diagnosis of GCA.
• Angiography of the arms is indicated in
patients with peripheral vascular insufficiency.
• FDG-PET imaging may be used to detect
large-vessel inflammation in GCA.
TREATMENT
ACUTE GENERAL Rx
• If there is clinical suspicion of GCA, treatment
should be initiated without waiting for results
of laboratory or imaging studies.
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GCA
suspicion low
No further
biopsy
• IV methylprednisolone (250-1000 mg for
1-3 days) is considered standard of care in
patients with severe clinical manifestations
such as visual loss from ischemic optic
neuropathy.
• Oral prednisone (1 mg/kg/day): high-dose
oral regimen should be continued at least
until symptoms resolve and ESR returns to
normal; usually 3 to 4 weeks after treatment
initiation. Steroid taper is very slow (10%-
20% per month) with monitoring of clinical
features as well as ESR and CRP. When
dose <10 mg/day, taper by 1 mg/month.
Treatment may last up to 2 yr or more.
Corticosteroids are the treatment of choice.
There is no evidence for the role of steroid-
sparing agents. Methotrexate, tocilizumab, and
cyclophosphamide may be considered in cases
of contraindications to or failure of cortico-
steroid therapy. Evidence for their efficacy is
limited.
DISPOSITION
With steroid therapy there is a dramatic
improvement of systemic symptoms, but not
vision in patients with ischemic optic neuropa-
thy. In one study only 4% of eyes improved in
both visual acuity and central visual field.
Management of flares: Repeat prednisone
induction if patient experiences severe flare.
If mild flare, increase prednisone by 10% to
20%.
REFERRAL
• Surgical or ophthalmologic referral for biopsy
of temporal artery.
• Rheumatology referral for long-term immu-
nosuppressive treatment management.
PEARLS &
CONSIDERATIONS
• Treatment of GCA should be started if there is
clinical suspicion of the disease. This usually
includes patients above the age of 50 pre-
senting with a severe headache and systemic
features that suggest GCA as well. Physicians
should not wait for laboratory or pathologic
confirmation before starting treatment as the
risk of visual loss increases.
• Temporal artery biopsy should be performed
as soon as possible, but within 2 weeks of
initiating treatment with steroids. The biopsy
may remain positive for two to six weeks
after initiation of corticosteroids.
• Treatment should not be withheld pending
temporal artery biopsy.
• Temporal artery biopsy may not be required
in patients with typical disease features
accompanied by characteristic ultrasound or
MRI findings.
COMMENTS
• The relation between polymyalgia rheumati-
ca and GCA is unclear, but the two frequently
coexist. They are considered to be different
points along the gradient or spectrum of the
same disease.
• Clinical picture rather than ESR should be
the prime yardstick for continuing pred-
nisone therapy. A rising ESR in a clinically
asymptomatic patient with normal hema-
tocrit should raise suspicion for alternate
explanations (e.g., infections, neoplasms).
• GCA is associated with a markedly increased
risk for the development of aortic aneurysm,
which is often a late complication and may
cause death. Annual chest radiograph in
chronic CGA patients has been suggested, as
well as emergent chest CT or MRI for clinical
suspicion.
• GCA is also associated with increased risk of
myocardial infarction, stroke, and peripheral
vascular disease.
• Coadministration of low dose aspirin (81 mg/
day) has been reported by some as effective
for further reduction of risk of blindness.
Additional trials may be needed before it can
be recommended as standard therapy.
SUGGESTED READINGS
Available at www.expertconsult.com.
RELATED CONTENT
Giant Cell Arteritis (Patient Information)
Temporal Arteritis (Patient Information)
AUTHORS: ARUN SWAMINATHAN, M.B.B.S., and
SACHIN KEDAR, M.B.B.S., M.D.
Giant Cell Arteritis 516.e1

SUGGESTED READINGS
Buttgereit F, et al.: Polymyalgia rheumatica and giant cell arteritis: a systematic
review, JAMA 315(22):2442–2458, 2016.
Hoffman GS: In the clinic: giant cell arteritis. Ann Intern Med ITCG7, Nov 1, 2016.
Tomasson G, et al.: Risk for cardiovascular disease early and late after a diagnosis
of giant-cell arteritis, Ann Int Med 160:73–80, 2014.
Weyand C, Goronzy JJ: Giant-cell arteritis and polymyalgia rheumatica, N Engl J
Med 371:50–57, 2014.

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For personal use only. No other uses without permission. Copyright ©2018. Elsevier Inc. All rights reserved.