Aortic Involvement in Recent-Onset Giant Cell
(Temporal) Arteritis: A Case–Control Prospective
Study Using Helical Aortic Computed
CHRISTIAN AGARD,1JACQUES-H. BARRIER,1BENOˆIT DUPAS,1THIERRY PONGE,1ALFRED MAHR,2
G´ERARD FRADET,3PASCAL CHEVALET,4AGATHE MASSEAU,1ERIC BATARD,1PIERRE POTTIER,1
BERNARD PLANCHON,1JEAN-MARIE BRISSEAU,1AND MOHAMED-A. HAMIDOU1
Objective. The prevalence of the involvement of large vessels in giant cell arteritis (GCA) is 3–13%. Aortitis is the most
serious complication of GCA. Computed tomodensitometric (CT) scan allows analysis of both the aortic wall and
endoluminal part of the aorta. Therefore, we conducted a study using CT scan to analyze aortic abnormalities in patients
with recent-onset GCA.
Methods. This prospective controlled study compared patients with biopsy-proven GCA with a matched control group
based on sex, age, and cardiovascular risk factors. During the 4-week period following diagnosis of GCA, patients
underwent an aortic CT scan. The aortic imaging results were blindly compared between both groups.
Results. From January 5, 1998 to January 11, 1999, 22 patients and 22 controls were screened by CT scan for aortic
involvement. Thickening of the aortic wall was more frequent among patients than controls (45.4% versus 13.6%; P ?
0.02). Aortic thickening (mean 3.3 mm) was located on the ascending part of the thoracic aorta in 22.7% of the patients,
with no evidence of thickening in the controls (P ? 0.05). Thickening of the abdominal aortic wall was noted in 27.3%
of the patients and none of the controls (P ? 0.02).
Conclusion. This study suggests that inflammatory aortic thickening, detected by CT scan, occurs frequently at the time
of diagnosis of GCA, and that this condition predominantly occurs on the ascending part of the aorta.
Giant cell (temporal) arteritis (GCA) is a middle and large-
vessel systemic vasculitis affecting patients over 50 years
of age (1,2). GCA is one of the most frequent systemic
vasculitides, with an annual incidence of the disease being
between 4.4 and 25 per 100,000 inhabitants over 50 years
of age (3–8).
The pathogenesis of GCA remains poorly understood.
The expression of HLA class II molecules could be an
indicator of predisposal to the disease (9,10). The activated
immune cells contribute to alterations of the superficial
temporal arterial wall, resulting in fragmentation of the
internal elastic lamina, reduction of media thickness, and
proliferation of smooth muscle cells, leading to an intimal
hyperplasia that directly correlates to ischemic manifesta-
In GCA, branches of the external carotid artery, espe-
cially the superficial temporal artery, are preferentially
involved by the inflammatory granulomatous process.
Most GCA cases are easy to diagnose, as the symptoms
generally include headache, fatigue, jaw claudication,
scalp hyperesthesia, polymyalgia rheumatica (PMR), ocu-
lar involvement, and marked inflammatory syndrome.
However, long delay in diagnosis of GCA does occur,
particularly when patients present with isolated fatigue,
unexplained inflammation, or fever of unknown origin
(12). Moreover, GCA may also involve extratemporal large
vessels, which were described approximately 60 years ago
(13–15). Such involvement can lead to an atypical presen-
1Christian Agard, MD, Jacques-H. Barrier, MD, Benoı ˆt
Dupas, MD, PhD, Thierry Ponge, MD, PhD, Agathe Masseau,
MD, Eric Batard, MD, Pierre Pottier, MD, PhD, Bernard
Planchon, MD, Jean-Marie Brisseau, MD, Mohamed-A.
Hamidou, MD, PhD: Ho ˆtel-Dieu, Nantes Cedex, France;2Al-
fred Mahr, MD, PhD: Cochin Hospital, Assistance Publique
Ho ˆpitaux de Paris, Paris, France;3Ge ´rard Fradet, MD: Cen-
tre Hospitalier, Luc ¸on Cedex, France;4Pascal Chevalet, MD:
Ho ˆpital Bellier, Nantes Cedex, France.
Address correspondence to Christian Agard, MD, Inter-
nal Medicine, Ho ˆtel-Dieu, Place Alexis Ricordeau, 44093
Nantes Cedex 01, France. E-mail: christian.agard@chu-
Submitted for publication March 9, 2007; accepted in
revised form September 21, 2007.
Arthritis & Rheumatism (Arthritis Care & Research)
Vol. 59, No. 5, May 15, 2008, pp 670–676
© 2008, American College of Rheumatology
tation of GCA (14,16–18). Involvement of the aorta repre-
sents the most serious complication, with high mortality
rates as it leads to aortic dissection or aortic aneurysm
rupture (19–21). In addition, aortic dissection is more
likely to be lethal if it involves the thoracic aorta (22). The
prevalence of aortitis during GCA has been retrospectively
evaluated to be 3–13% (18,19). Aortitis can be present at
the initial stage of GCA, and may dramatically reveal the
presence of the disease (23). However, aortic complica-
tions, such as aortic aneurysms, may also occur many
years after the diagnosis of GCA.
Some authors recommend yearly chest radiograph and
transthoracic echocardiogram to detect signs suggesting a
thoracic aortic aneurysm in patients with GCA (24,25).
Patients with aortic insufficiency murmur could be con-
sidered at a higher risk for GCA, and could be screened for
aortic aneurysm with an abdominal echocardiograph and a
thoracic computed tomodensitometric (CT) scan, or mag-
netic resonance imaging, at the time of diagnosis (25).
However, to date, no prospective systematic studies
about aortic description in GCA are available. Moreover,
no predictive factors of aortic involvement have ever been
prospectively asserted in GCA.
We, and others, have already underlined the interest in
aortic CT scan to search for morphologic abnormalities of
the aorta (22,25–27). Indeed, CT scan is a rapid, noninva-
sive, and reproducible technique that allows description
of the aortic wall, as much as to the endoluminal part of
the aorta. Therefore, we conducted a prospective imaging
study using helical aortic CT scan to screen patients with
GCA at the time of diagnosis for aortic abnormalities.
PATIENTS AND METHODS
Study design. This single-center prospective study was
conducted in the internal medicine department of the
University Hospital in Nantes, France. Patients with GCA
seen in this unit are typically derived from the local pop-
ulation. Twenty-two patients with de novo GCA were con-
secutively included during an 18-month period, from Jan-
uary 5, 1998 to January 11, 1999. Each GCA patient was
matched with a control on sex, age, and cardiovascular
risk factors. Controls were randomly chosen from a pool of
patients without GCA, PMR, or unexplained inflammatory
syndrome who underwent thoracoabdominal CT scan for
the followup of lymphoma or melanoma. Both of these
diseases were chosen because of their high prevalence,
their frequent CT scan evaluation, and the absence of any
known correlation with aortitis and/or aortic aneurysm.
Controls were also derived from the same local population
as the GCA patients. This study was approved by the local
ethics review committee and each GCA patient was asked
to provide written consent to participate.
Inclusion criteria. The inclusion criteria were as fol-
lows: ?55 years of age, histologically proven GCA with a
duration no longer than 4 weeks since diagnosis, and
fulfillment of at least 3 of the 5 1990 American College of
Rheumatology criteria for the diagnosis of GCA (28). His-
tologic proof of GCA had to be assessed by pathologic
analysis of a superficial temporal artery sample, showing
an inflammatory infiltrate of the vascular wall, with or
without giant cells, and associated with complete or par-
tial destruction of the elastic internal lamella.
Exclusion criteria. The following exclusion criteria
were used: allergy to iodine, absence of patient consent to
participate in the study, renal failure defined by a creat-
ininemia level twice controlled and ?1.58 mg/dl, and
infectious vasculitis or systemic vasculitis (other than
Collection of patient data. The following cardiovascu-
lar risk factors were investigated: family history of docu-
mented myocardial infarction or stroke, hypercholesterol-
emia, diabetes mellitus (cutoff 126 mg/dl), arterial
hypertension (cutoff 140/90 mm Hg), tobacco consump-
tion (?10 pack-years), and excess weight as defined by a
body mass index ?28 kg/m2. From these data, a 7-point
cardiovascular risk score was calculated, with each of the
cardiovascular risk factors listed above counted as 1 point.
The presence of peripheral ischemic signs was also in-
vestigated, including painful claudication, or pain at rest
in the upper or lower limbs, and limb paresthesias. Clini-
cal examination systematically looked for peripheral
pulses, vascular murmur, asymmetrical pulse or blood
pressure, and the sign of DeBakey.
The following biologic parameters were measured in
each patient before corticosteroid therapy introduction:
C-reactive protein (CRP) level (mg/liter), erythrocyte sedi-
mentation rate (ESR; first hour, Westergren method), fibrin
(gm/liter), hemoglobin (gm/dl), platelet count, total num-
ber of white blood cells, neutrophils and eosinophils, se-
rum urea (mmoles/liter), creatininemia (mg/liter), fasting
glycemia (mmoles/liter), and total cholesterolemia (gm/
Detection of anticardiolipin (aCL) antibodies (by stan-
dardized immunoenzymatic test) was performed in 15 of
the patients. IgG aCL antibody values ?10 IgG phospho-
lipid units were considered to be a positive result.
Control group. The control group was matched on sex
and age to the patient group. Cardiovascular risk factors
were investigated for the control group and the same score
was calculated as for the GCA patient group; the mean
score was compared between the 2 groups. Individuals
from the control group did not receive any chemotherapy
in the 3 months before CT scan was performed. Investiga-
tors ensured that patients from the control group did not
have long-term corticosteroid therapy, GCA, other sys-
temic vasculitis, or PMR. In addition, investigators verified
that none of the controls developed GCA during the year
following the study. Patients in the control group were
consecutively included at random during a 6-week period
from a group of patients who underwent thoracoabdomi-
nal CT scan for the followup of either lymphoma or mel-
anoma. The helical CT scan protocol was rigorously fol-
lowed the same as it was for the GCA patients.
Aortic Involvement in GCA671
Aortic helical CT scan protocol. Each helical CT scan of
the aorta was performed in the department of radiology at
the University Hospital in Nantes with a Philips A.V.E.1
model (Phillips, Rotterdam, The Netherlands). A standard-
ized protocol was previously defined noting the following
parameters: CT scan section thickness 10 mm, speed of
movement of patient support 10 mm/second, intravenous
injection of iodine contrast product (iodine concentration
300 mg/ml) 2 ml/kg with a flow equal to 3 ml/second and
stopped 20 seconds before the end of the acquisition of the
image, and reconstruction (1 second per CT scan section)
with an intersection distance of 8 mm. A prophylactic
intravenous hydration (1.5 liter salt serum) during 24
hours was initiated 4 hours before the CT scan, and serum
creatinine was checked 48 hours after the CT scan.
In the 4 weeks following diagnosis of GCA, an aortic
helical CT scan was performed in each patient included in
the study, and the same evaluation was performed in pa-
tients from the control group. The CT scans were analyzed
by the same diagnosis-blinded radiologist (BD).
Analysis of the aortic CT scans noted the following
criteria: presence or absence of 1 (or more) aortic aneu-
rysm, presence or absence of 1 (or more) aortic ectasia,
aspect of aortic dissection, maximal wall thickness of the
aorta, and presence of aortic stenosis, aortic thrombus, or
lesions suggesting aortic atherosclerosis. Aortic wall thick-
ness ?2 mm was considered aortic thickening. These dif-
ferent items were separately analyzed on the thoracic and
Aneurysm was defined as aortic dilatation with a loss of
wall parallelism, according to the New York Heart Asso-
ciation (29). Aortic ectasia was determined by the radiol-
ogist to be an abnormal dilatation, without loss of wall
parallelism. In the absence of aortic atherosclerosis, aneu-
rysm, ectasia, dissection, or thickening, the aorta was con-
sidered to be normal.
The following criteria were required to define a suspi-
cion of CT scan–specific aortitis among GCA patients: a
thickening of the aortic wall (without adjacent atheroscle-
rotic plaque) and/or an aortic aneurysm or ectasia, except
in patients diagnosed for more than 2 years.
Statistical analysis. Categorical variables were ex-
pressed as percentages and compared using chi-square
tests or Fisher’s exact tests when any of the expected cell
counts of a 2 ? 2 table was less than 5. Comparisons of
quantitative variables were performed using Student’s t-
tests. All tests were 2-sided and a P value less than 5% was
considered to be statistically significant. All statistical ana-
lyses were performed using Epiinfo software, version 1.0.3
(Centers for Disease Control and Prevention, Atlanta, GA).
Clinical and biologic description of the GCA patient
group. Twenty-two patients with biopsy-proven GCA
were included in this study, with a total of 17 women and
5 men (female:male sex ratio 3.4). All patients were white.
The mean age of the GCA group was 73.7 years (range
61–86 years) and the mean age of the control group (also
including 17 women and 5 men) was 71.2 years (range
60–93 years), which was not statistically different from the
mean age of the patient group. The cardiovascular risk
factor score was 2.45 in the patient group and 2.36 in the
control group (P ? 0.75); therefore, both groups were
matched for age, sex, and cardiovascular risk factors.
Clinical and biologic presentation of the GCA patients is
summarized in Table 1. Presenting signs included the
following: fatigue (82%), fever (77%), headache (73%),
weight loss (59%), hyperesthesia of the scalp (36%), PMR
(32%), jaw claudication (23%), and ocular involvement
(13.6%). Ten patients (45.5%) presented with clinical
signs suggesting involvement of large vessels at the time of
GCA diagnosis; these signs were determined to be the
following: claudication of an upper limb (n ? 4), abolition
of peripheral pulses (n ? 4), stroke contemporary to GCA
(n ? 2), history of stroke (n ? 1), or carotid bruit (n ? 1).
One 74-year-old man had a thoracic aortic aneurysm dis-
covered 20 years earlier. None of the patients had abdom-
inal pulsatile masses or abdominal bruit. Eight patients
(36%) had previously diagnosed aortic valvulopathy: aor-
tic stenosis in 6 cases and grade 2 aortic incompetence in
2 cases. One patient had evidence of aortitis, suggested by
occurrence of fatal dissection of a thoracoabdominal aneu-
rysm 10 days after starting corticosteroid therapy for GCA.
Table 1. Clinical and biologic presentation of giant cell
arteritis patients at inclusion (n ? 22)*
Age, mean (range) years
Hyperesthesia of the scalp
Thoracic aortic aneurysm†
Abdominal aortic aneurysm
History of stroke
Upper limb ischemia at inclusion
Lower limb ischemia at inclusion
History of carotid stenosis
Biologic results, median (range)
ESR, mm/hour (normal ?10)
CRP, mg/liter (normal ?5)
Fibrinogen, gm/liter (normal 2–4)
Hemoglobin, gm/dl (normal 12–16)
Platelets, ? 109/liter (normal 150–400)
White blood cells, ? 109/liter
Neutrophils, ? 109/liter (normal 3–6)
* Values are the number (percentage) unless otherwise indicated.
ESR ? erythrocyte sedimentation rate; CRP ? C-reactive protein.
† One patient had a 20-year preexisting thoracic aortic aneurysm.
672 Agard et al
Median ESR and CRP values were 80 mm/hour and 116
mg/liter, respectively. The median hemoglobin value was
11.1 gm/dl and 16 of the patients had anemia. The median
platelet count was 355.5 ? 109/liter, and the median total
white blood cell and neutrophil counts were 9.25 ? 109/
liter and 6.54 ? 109/liter, respectively. Fifteen patients
were screened for aCL antibodies and 5 of them (42%)
were positive. None of the patients had a history of deep
vein thrombosis or pulmonary embolism.
Analysis of aortic helical CT. The aortic helical CT
procedure was well tolerated without occurrence of al-
lergy or renal failure. Abnormal aortic CT results were
more frequent among the GCA patients than the controls
(82% versus 68%). One patient presented with a dissect-
ing thoracic aortic aneurysm that revealed GCA. We found
no differences between the 2 groups in the frequency of
aortic ectasia and aneurysm. Aortic atherosclerotic lesions
were equally distributed among the patients and controls
(72.7% versus 68.2%). Abnormal thickening of the aortic
wall (Figure 1) was more frequent among GCA patients
than controls (45.4% versus 13.6%; P ? 0.02).
Analysis of the helical CT scan of the thoracic aorta is
presented in Table 2. Three patients with GCA (13.6%)
and 1 control (4.5%) presented with thoracic aortic aneu-
rysm (Figure 2). One of the GCA patients had a preexisting
thoracic aortic aneurysm. Aortic ectasia and/or aneurysm
was found in 22.7% of the GCA patients versus 9% of the
controls, but this difference was not statistically signifi-
cant (P ? 0.41). A thickened thoracic aortic wall was noted
in 10 of the patients with GCA (45.4%), whereas only
13.6% of controls had such a thickening (P ? 0.02). The
thickening was located on the ascending part of the tho-
racic aorta for 22.7% of the GCA patients, with no thick-
ening seen in the controls (P ? 0.05). The mean thoracic
Figure 1. Examples of aortic computed tomodensitometric scan
findings noted in 2 different giant cell arteritis (GCA) patients (A
and B) and controls matched on age, sex, and cardiovascular risk
factors (C and D). Thickenings of the aortic wall (arrows) were
only noted in GCA patients. Thickenings measuring 5 mm were
located on the descendant aorta (A) or on the abdominal aorta (B).
Table 2. Comparison of helical computed tomodensitometric findings noted on the
thoracic aorta between 22 patients with giant cell arteritis and 22 controls*
Normal thoracic aorta
Aortic thoracic aneurysm
Aortic thoracic aneurysm and/or ectasia
Thickening of the thoracic aortic wall
Thickening of the ascending thoracic aortic wall
Mean thickness of the aortic wall (mm)
Atherosclerosis of the thoracic aorta
* Values are the number (percentage) unless otherwise indicated.
† One patient had a 20-year preexisting thoracic aortic aneurysm.
Figure 2. Examples of aortic computed tomodensitometric scan
findings noted in 2 different giant cell arteritis (GCA) patients (A
and B) and controls matched on age, sex, and cardiovascular risk
factors (C and D). Aneurysms (An) of the ascending part of the
thoracic aorta were seen in GCA patients. The aortic aneurysm of
one patient (B) was associated with a 3-mm thickening of the
entire thoracic aorta (arrow).
Aortic Involvement in GCA 673
aortic thickening in the GCA patients was 3.33 mm. The
thickening comprised half of the aortic circumference in 3
of the cases, and was located on a single side of the vessel
in the 7 other cases. Among the controls, 3 patients pre-
sented with thoracic aortic thickening (at 5 mm, 2 mm, and
4 mm), which exclusively comprised the descendent part
of the aorta. Thoracic aortic abnormalities, suggesting ath-
erosclerosis (arterial wall calcifications), were seen in 50%
of the GCA patients but in only 27% of the controls.
Analysis of the helical CT scan results of the abdominal
aorta is presented in Table 3. One of the GCA patients (the
patient who revealed his disease by a fatal thoracic aortic
dissection) did not undergo abdominal CT. None of the 21
GCA patients presented with abdominal aortic aneurysm
and/or ectasia. One patient presented abnormalities sug-
gesting inflammatory periaortitis of the abdominal aorta.
Six of the GCA patients (27.3%) had a thickening of the
abdominal aortic wall, measured at 2 mm (2 cases), 3 mm
(2 cases), and 4 mm (2 cases). None of the controls pre-
sented such thickenings, and the difference between the
groups was statistically significant (P ? 0.02). In addition,
lesions of atherosclerosis of the abdominal aorta were vis-
ible in 15 of the patients (68%) and 14 of the controls
In our study, a total of 10 patients (45.5%) had CT scan
results demonstrating suspicion of specific aortitis. The
comparison between these 10 patients and the other 12
GCA patients did not show any significant differences in
the clinical and biologic presentation. Nevertheless, we
observed that patients with suspicion of aortitis were more
likely to have fever (90% versus 66%), aortic incompe-
tence (20% versus 0%), history of stroke (20% versus 0%),
and ischemia of the upper limbs (30% versus 8.3%) and
the lower limbs (40% versus 8.3%).
Our present study describes the morphologic abnormali-
ties of the aorta detected in patients with recent-onset GCA
who underwent aortic helical CT. To our knowledge, this
is the first study to report a prospective and comparative
screening of aortic lesions in the acute phase of GCA. In
our experience, aortic abnormalities were found in 16
(73%) of the 22 GCA patients evaluated. The main abnor-
mality observed was a thickening of the aortic wall, lo-
cated in the thoracic area of the aorta (45.4% in GCA
patients versus 13.6% in controls; P ? 0.02) and the ab-
dominal part of the aorta (27.3% versus 0%; P ? 0.01).
These findings did not seem to be related to atherosclero-
sis. Although the GCA patients had more frequent athero-
sclerotic lesions than the controls, this difference was not
statistically significant. Moreover, we did not find more
severe atherosclerotic lesions among the GCA patients
(data not shown). When present on the thoracic aorta, such
thickening was also present on the ascending part of the
aorta in 5 GCA patients, whereas none of the non-GCA
controls had such an abnormality. This finding is consis-
tent with the notion that GCA inflammation has an elective
tropism for the ascending part of the aorta, as noted by
many authors (16–20,22,30). Taken together, our results
indicate that aortic thickenings may be present in the early
acute phase of GCA. Although they cannot be histologi-
cally documented, the differences between GCA patients
and controls suggest that some of these thickenings are
possibly acute aortic inflammatory lesions of GCA.
Aortitis during GCA is potentially serious. It can reveal
GCA in an acute manner and can be responsible for sud-
den death (18,19,31,32). A body of evidence supports the
notion that the life expectancy of patients with GCA is
similar to that of the age-matched population (4,33,34).
However, it is likely that patients with aortic involvement
could be predisposed to fatal complications, such as aortic
dissection (25). Uddhammar et al have reported an in-
creased mortality due to cardiovascular disease during
GCA (35). Thus, aortitis may represent the cause of sudden
death, as seen in 12 of the 35 cases reported by Lie (20). A
retrospective study recently found no differences in sur-
vival between a group of 46 patients with GCA with large-
artery complications and a group of 122 patients with GCA
without large-artery involvement (36). In our series, 1 pa-
tient died suddenly after the rupture of an aortic thoracic
aneurysm, illustrating the seriousness of such locations.
Of note, this case has been separately published (23).
Fortunately, aortitis appears to be rare. A retrospective
study spanning 50 years found 30 incident cases (18%) of
aortic aneurysm and/or dissection among 168 patients
with GCA (22). In addition, patients with GCA may de-
velop aortic arch syndrome in approximately 4–15% of
cases (18,37,38). GCA seems to be a condition that favors
the occurrence of aortic aneurysm. When compared with
the age- and sex-matched population, patients with GCA
from Olmsted County, Minnesota, were 17.3 times more
likely to develop a thoracic aortic aneurysm and 2.4 times
more likely to develop an abdominal aortic aneurysm (24).
The retrospective analysis of the 96 GCA patients noted
that 16 (17%) of the patients either had an aortic aneurysm
at the time of diagnosis or developed one later (24). A
retrospective study in Spain found that 7.6% of 210 pa-
tients with GCA developed thoracic aortic aneurysms,
while abdominal aneurysms were seen in 2.9% of the
patients (30). GCA is thought to be the main cause of GCA
among patients over 60 years of age (39). As suggested in
our study, silent inflammatory aortitis can be present at the
time of GCA diagnosis. This was also supported by O¨st-
berg, who noted aortic histologic changes in 2–7% of cases
Table 3. Comparison of helical computed
tomodensitometric findings noted on the abdominal
aorta between 22 patients with giant cell arteritis and
Normal abdominal aorta
Abdominal aortic aneurysm
Thickening of the abdominal
Mean thickness of the aortic
Atherosclerosis of the
15 (68.2) 14 (63.6) 0.75
* Values are the number (percentage) unless otherwise indicated.
674Agard et al
on necropsic analysis (40). Therefore, many authors con-
sider that large-vessel involvement in GCA is more fre-
quent than previously thought (2,25,30), and moreover,
that most patients with GCA have aortic inflammation,
mostly asymptomatic, at some point during their disease
(22,41). Although we believe that some of the thickenings
we observed on the aortic wall were probably due to active
GCA, the clinical significance of these thickenings remains
to be established. As such, we are unable to appreciate
whether they are real predisposing factors for aneurysm
formation. However, we observed aortic thickenings in the
ascending part of the aorta, which is also the preferential
site of aneurysm in patients with GCA.
In the present work, aortic aneurysms were more fre-
quently observed among the GCA patients than the con-
trols, but the difference was not found to be statistically
significant. Nevertheless, the trend observed was in agree-
ment with previous data (22,24,30). According to Evans et
al, in 80% of cases, aneurysm occurs several years after
diagnosis of GCA, with a mean delay of 7 years (24). Aortic
aneurysm may be detected at the time of diagnosis of GCA,
and our group has recently reported that small abdominal
aortic aneurysm surrounded by a hypoechoic halo could
be detected using ultrasonography (42).
Despite its seriousness, no predictive factors for aortic
involvement in GCA have ever been clearly defined. Arte-
rial hypertension, severe inflammatory response at the
time of diagnosis of GCA, persistent chronic inflammatory
response, or frequent relapses could be predictors for an
aortic aneurysmal disease (30). Hyperlipidemia and coro-
nary artery disease may also be associated with aortic
aneurysm and/or dissection (22). In our study, a total of 10
patients (45.5%) had aortic complications that were poten-
tially related to GCA. When these 10 patients were com-
pared with the 12 patients without suspicion of GCA-
related aortic lesions, we were not able to find any
significant differences in the clinical presentation, the in-
flammatory response, the presence of aCL, or the HLA
class II alleles (data not shown). However, a study includ-
ing a larger number of patients would be of interest to
answer this question.
We believe that our results should be interpreted with
caution, because the present study may have potential bias
and limitations. First, patients referred to our hospital may
have more frequent atypical signs of GCA, including large-
vessel involvement. However, a large majority of our pa-
tients (73%) had cephalalgia, which is considered the
most typical sign of GCA. Second, patients from the con-
trol group were not healthy volunteers. We cannot exclude
that their disease (melanoma or lymphoma), or their treat-
ment, might have induced any aortic changes. However,
there are no data in the literature to clearly support this
hypothesis. The main limitation of the present study is
related to the pathogenetic significance of the aortic wall
thickenings that we observed. Although almost half of the
GCA patients had such a thickening, the incidence of
aortic aneurysm in GCA is obviously lower. Therefore, it is
not validated that each aortic wall thickening is a sole
predisposing factor for aneurysm formation. However, this
raises an interesting question that has to be answered with
long-term followup prospective studies. Until these stud-
ies, we do not recommend the screening of all patients
with GCA with aortic CT scan. In contrast, we perform an
aortic CT scan when clinical signs suggest aortic involve-
ment, such as aortic arch syndrome or aortic incompetence
murmur. Other imaging techniques might be useful to
appreciate an aortic involvement in patients with GCA.
The abdominal aorta can be investigated with ultrasonog-
raphy, and recent studies found a hypoechoic halo, inter-
preted as an inflammatory edema, around the temporal or
large vessels (42–45). However, ultrasonography cannot
explore the aorta at the thoracic stage, which is the pref-
erential location of aortitis in patients with GCA. Magnetic
resonance imaging and positron emission tomography
may also be of interest to assess and follow inflammatory
aortic wall changes (41,46).
In conclusion, this first, prospective, aortic CT scan
study in patients with recent-onset, biopsy-proven GCA
suggests that specific inflammatory aortic thickenings do
frequently exist at the time of diagnosis of GCA. These
abnormalities are predominantly located on the ascending
part of the aorta, which is concordant with the data present
in the literature concerning aortic inflammation in GCA.
Whether aortic thickenings are related to the inflammatory
processes leading to vascular wall injury responsible for
late-onset aneurysmal disease remains to be established.
Further prospective imaging studies including patients
with CT scan aortic abnormalities at the initial stage of
GCA are required to answer this question.
Dr. Agard had full access to all of the data in the study and takes
responsibility for the integrity of the data and the accuracy of the
Study design. Agard, Barrier, Dupas, Planchon, Hamidou.
Acquisition of data. Agard, Dupas, Ponge, Fradet, Chevalet, Ba-
tard, Pottier, Brisseau, Hamidou.
Analysis and interpretation of data. Agard, Barrier, Dupas, Mas-
Manuscript preparation. Agard, Barrier, Ponge, Hamidou.
Statistical analysis. Agard, Mahr.
1. Hunder GG. Giant cell arteritis in polymyalgia rheumatica.
Am J Med 1997;102:514–6.
2. Weyand CM, Goronzy JJ. Giant-cell arteritis and polymyalgia
rheumatica. Ann Intern Med 2003;139:505–15.
3. Machado EB, Michet CJ, Ballard DJ, Hunder GG, Beard CM,
Chu CP, et al. Trends in incidence and clinical presentation of
temporal arteritis in Olmsted County, Minnesota, 1950–1985.
Arthritis Rheum 1988;31:745–9.
4. Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback LR.
Temporal arteritis: a 25-year epidemiologic, clinical, and
pathologic study. Ann Intern Med 1978;88:162–7.
5. Barrier J, Pion P, Massari R, Peltier P, Rojouan J, Grolleau JY.
Epidemiologic approach to Horton’s disease in the depart-
ment of Loire-Atlantique: 110 cases in 10 years (1970-1979).
Rev Med Interne 1982;3:13–20. In French.
6. Nordborg E, Bengtsson BA. Epidemiology of biopsy-proven
giant cell arteritis (GCA). J Intern Med 1990;227:233–6.
7. Salvarani C, Macchioni PL, Tartoni PL, Rossi F, Baricchi R,
Castri C, et al. Polymyalgia rheumatica and giant cell arteritis:
a five year epidemiologic and clinical study in Reggio Emilia,
Italy. Clin Exp Rheumatol 1987;5:205–12.
8. Boesen P, Sorensen SF. Giant cell arteritis, temporal arteritis,
Aortic Involvement in GCA675
and polymyalgia rheumatica in a Danish county: a prospec- Download full-text
tive investigation, 1982–1985. Arthritis Rheum 1987;30:
9. Barrier J, Bignon JD, Soulillou JP, Grolleau JY. Increased prev-
alence of HLA-DR4 in giant-cell arteritis. N Engl J Med 1981;
10. Weyand CM, Hunder NN, Hicok KC, Hunder GG, Goronzy JJ.
HLA–DRB1 alleles in polymyalgia rheumatica, giant cell ar-
teritis, and rheumatoid arthritis. Arthritis Rheum 1994;37:
11. Weyand CM, Goronzy JJ. Arterial wall injury in giant cell
arteritis [review]. Arthritis Rheum 1999;42:844–53.
12. Hamidou MA, Batard E, Trewick D, Masseau A, Moreau A,
Agard C, et al. Silent versus cranial giant cell arteritis: initial
presentation and outcome of 50 biopsy-proven cases. Eur
J Intern Med 2005;16:183–6.
13. Gilmour JR. Giant cell chronic arteritis. J Pathol 1941;53:263–
14. Cooke WT, Cloake PC, Govan AD, Colbeck JC. Temporal
arteritis: a generalized vascular disease. QJM 1946;15:47–75.
15. Jennings GH. Arteritis of the temporal vessels. Lancet 1938;
16. Hamrin B, Jonsson N, Landberg T. Involvement of large ves-
sels in polymyalgia arteritica. Lancet 1965;1:1193–6.
17. Ostberg G. Morphological changes in the large arteries in
polymyalgia arteritica. Acta Med Scand Suppl 1972;533:135–
18. Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery
involvement in giant cell (temporal) arteritis. Ann Intern Med
19. Evans JM, Bowles CA, Bjornsson J, Mullany CJ, Hunder GG.
Thoracic aortic aneurysm and rupture in giant cell arteritis: a
descriptive study of 41 cases. Arthritis Rheum 1994;37:1539–
20. Lie JT. Aortic and extracranial large vessel giant cell arteritis:
a review of 72 cases with histopathologic documentation.
Semin Arthritis Rheum 1995;24:422–31.
21. Liu G, Shupak R, Chiu BK. Aortic dissection in giant-cell
arteritis. Semin Arthritis Rheum 1995;25:160–71.
22. Nuenninghoff DM, Hunder GG, Christianson TJ, McClelland
RL, Matteson EL. Incidence and predictors of large-artery
complication (aortic aneurysm, aortic dissection, and/or
large-artery stenosis) in patients with giant cell arteritis: a
population-based study over 50 years. Arthritis Rheum 2003;
23. Agard C, Ponge T, Fradet G, Baron O, Sagan C, Masseau A, et
al. Giant cell arteritis presenting with aortic dissection: two
cases and review of the literature. Scand J Rheumatol 2006;
24. Evans JM, O’Fallon WM, Hunder GG. Increased incidence of
aortic aneurysm and dissection in giant cell (temporal)
arteritis: a population-based study. Ann Intern Med 1995;122:
25. Bongartz T, Matteson EL. Large-vessel involvement in giant
cell arteritis. Curr Opin Rheumatol 2006;18:10–7.
26. Agard C, Ponge T, Hamidou M, Barrier J. Role for vascular
investigations in giant cell arteritis. Joint Bone Spine 2002;
27. Herve F, Choussy V, Janvresse A, Cailleux N, Levesque H,
Marie I. Aortic involvement in giant cell arteritis: a prospec-
tive follow-up of 11 patients using computed tomography.
Rev Med Interne 2006;27:196–202. In French.
28. Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP,
Calabrese LH, et al. The American College of Rheumatology
1990 criteria for the classification of giant cell arteritis. Ar-
thritis Rheum 1990;33:1122–8.
29. The Criteria Committee of the New York Heart Association.
Nomenclature and criteria for diagnosis of diseases of the
heart and great vessels. 9th ed. Boston: Little, Brown and Co;
30. Gonzalez-Gay MA, Garcia-Porrua C, Pineiro A, Pego-Reigosa
R, Llorca J, Hunder GG. Aortic aneurysm and dissection in
patients with biopsy-proven giant cell arteritis from north-
western Spain: a population-based study. Medicine (Balti-
31. Save-Soderbergh J, Malmvall BE, Andersson R, Bengtsson BA.
Giant cell arteritis as a cause of death: report of nine cases.
32. Richardson MP, Lever AM, Fink AM, Dixon AK, Hazleman
BL. Survival after aortic dissection in giant cell arteritis [let-
ter]. Ann Rheum Dis 1996;55:332–3.
33. Barrier J, Tournemaine N, Maulaz D, Mainguy-Fonteneau B,
Rojouan J, Potel G, et al. Outcome, treatment and prognosis of
Horton’s disease. Ann Med Interne (Paris) 1983;134:428–35.
34. Bengtsson BA, Malmvall BE. Prognosis of giant cell arteritis
including temporal arteritis and polymyalgia rheumatica.
Acta Med Scand 1981;209:337–45.
35. Uddhammar A, Eriksson AL, Nystrom L, Stenling R, Ran-
tapaa-Dahlqvist S. Increased mortality due to cardiovascular
disease in patients with giant cell arteritis in northern Swe-
den. J Rheumatol 2002;29:737–42.
36. Nuenninghoff DM, Hunder GG, Christianson TJ, McClelland
RL, Matteson EL. Mortality of large-artery complication (aor-
tic aneurysm, aortic dissection, and/or large-artery stenosis)
in patients with giant cell arteritis: a population-based study
over 50 years. Arthritis Rheum 2003;48:3532–7.
37. Ninet JP, Bachet P, Dumontet CM, Du Colombier PB, Stewart
MD, Pasquier JH. Subclavian and axillary involvement in
temporal arteritis and polymyalgia rheumatica. Am J Med
38. Perruquet JL, Davis DE, Harrington TM. Aortic arch arteritis
in the elderly: an important manifestation of giant cell arteri-
tis. Arch Intern Med 1986;146:289–91.
39. Rojo-Leyva F, Ratliff NB, Cosgrove DM 3rd, Hoffman GS.
Study of 52 patients with idiopathic aortitis from a cohort of
1,204 surgical cases. Arthritis Rheum 2000;43:901–7.
40. Ostberg G. An arteritis with special reference to polymyalgia
arteritica. Acta Pathol Microbiol Scand Suppl 1973;237 Suppl
41. Blockmans D, Stroobants S, Maes A, Mortelmans L. Positron
emission tomography in giant cell arteritis and polymyalgia
rheumatica: evidence for inflammation of the aortic arch.
Am J Med 2000;108:246–9.
42. Agard C, Hamidou MA, Said L, Ponge T, Connault J, Chevalet
P, et al. Screening of abdominal aortic involvement using
Doppler sonography in active giant cell (temporal) arteritis at
the time of diagnosis: a prospective study of 30 patients. Rev
Med Interne 2007;28:363–70. In French.
43. Schmidt WA, Kraft HE, Vorpahl K, Volker L, Gromnica-Ihle
EJ. Color duplex ultrasonography in the diagnosis of temporal
arteritis. N Engl J Med 1997;337:1336–42.
44. Schmidt WA, Kraft HE, Borkowski A, Gromnica-Ihle EJ. Color
duplex ultrasonography in large-vessel giant cell arteritis.
Scand J Rheumatol 1999;28:374–6.
45. Schmidt WA, Blockmans D. Use of ultrasonography and
positron emission tomography in the diagnosis and assess-
ment of large-vessel vasculitis. Curr Opin Rheumatol 2005;
46. Narvaez J, Narvaez JA, Nolla JM, Sirvent E, Reina D, Valverde
J. Giant cell arteritis and polymyalgia rheumatica: usefulness
of vascular magnetic resonance imaging studies in the diag-
nosis of aortitis. Rheumatology (Oxford) 2005;44:479–83.
676 Agard et al