r e v c o l o m b r e u m a t o l .

2 0 2 1;2 8(3):203–212

www.elsevier.es/rcreuma

Topic Review

Temporal artery Doppler ultrasound in patients

with giant cell arteritis: State of the art and
systematic review of the literature夽

Tomás Urrego-Callejas a,b,∗ , Daniel Jaramillo-Arroyave a,c,d,e ,

Adriana-Lucía Vanegas-García a,e,f , Carlos Horacio Muñoz-Vahos a,c,e,f ,
Maribel Plaza Tenorio f
a Systemic Vasculitis Study Group, Faculty of Medicine, Universidad de Antioquia, Medellín, Colombia
b Cellular immunology and Immunogenetics Group, Faculty of Medicine, Universidad de Antioquia, Medellín, Colombia
c University IPS, Health Services, Faculty of Medicine, Universidad de Antioquia, Medellín, Colombia
d Epidemiology and Biostatistics Group, Faculty of Medicine, CES University, Medellín, Colombia
e Group of Rheumatology, Faculty of Medicine, Universidad de Antioquia, Medellín, Colombia
f San Vicente Hospital Foundation, Medellín, Colombia

a r t i c l e i n f o a b s t r a c t

Article history: Giant cell arteritis is a vasculitis that predominantly affects large caliber vessels, and usually
Received 2 July 2020 appears in people over 50 years-old. Its clinical presentation includes headache, hearing
Accepted 20 August 2020 impairment, or polymyalgia rheumatica-like symptoms. In its most severe form, it can cause
Available online 21 July 2021 uni- or bilateral vision loss secondary to arteritic ischemic optic neuropathy. Currently, the
gold standard for its diagnosis is the temporal artery biopsy, a procedure that is not harmless
Keywords: and may have complications such as infection, nerve injury, bleeding, among others. Among
Ultrasound non-invasive diagnostic methods, the ultrasound and temporal artery Doppler have gained
Doppler a predominant role in the diagnosis of giant cell arteritis, as it is a benign test with no
Vasculitis adverse effects. Through strategies such as “fast-track” clinics, supported by this diagnostic
Giant cell arteritis method, a reduction has been achieved in ischemic complications of the disease.
© 2020 Asociación Colombiana de Reumatologı́a. Published by Elsevier España, S.L.U. All
rights reserved.

PII of original article: S0121-8123(20)30141-9

夽
Please cite this article as: Urrego-Callejas T, Jaramillo-Arroyave D, Vanegas-García A-L, Muñoz-Vahos CH, Tenorio MP. Ultrasonido
Doppler de arterias temporales en pacientes con arteritis de células gigantes: estado del arte y revisión sistemática de la literatura. Rev
Colomb Reumatol. 2021;28:203–212.
∗
Corresponding author.
E-mail address: [email protected] (T. Urrego-Callejas).
2444-4405/© 2020 Asociación Colombiana de Reumatologı́a. Published by Elsevier España, S.L.U. All rights reserved.
204 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212

Ultrasonido Doppler de arterias temporales en pacientes con arteritis de

células gigantes: estado del arte y revisión sistemática de la literatura

r e s u m e n

Palabras clave: La arteritis de células gigantes es una vasculitis que afecta de manera predominante a vasos
Ultrasonido de gran calibre y aparece en personas mayores de 50 años. Su presentación clínica incluye
Doppler cefalea, alteraciones auditivas o síntomas similares a polimialgia reumática. En su forma
Ecografía más grave puede causar pérdida de visión uni o bilateral, secundaria a neuropatía óptica
Vasculitis isquémica de tipo arterítico. En la actualidad, el estándar de referencia para su diagnóstico
Arteritis de células gigantes es la biopsia de arterias temporales, procedimiento que no es inocuo y que puede tener como
complicaciones infección, lesión nerviosa o sangrado, entre otras. Entre las técnicas no inva-
sivas de diagnóstico, el ultrasonido y el Doppler de arterias temporales han tomado un rol
cada vez más importante en el diagnóstico de esta patología, dado que son pruebas benignas,
con nulos efectos adversos, y a través de estrategias como las clínicas fast-track apoyadas
en este método diagnóstico se ha logrado la reducción de complicaciones isquémicas de la
enfermedad.
© 2020 Asociación Colombiana de Reumatologı́a. Publicado por Elsevier España, S.L.U.
Todos los derechos reservados.

in 10% of patients with a previous diagnosis of polymyal-

Giant cell arteritis, what is it and what is the gia rheumatica.6 Early and timely diagnosis and treatment of
importance of timely diagnosis and treatment? patients with GCA is essential to prevent serious vascular com-
plications of the disease, particularly blindness. Even though
Giant cell arteritis (GCA) is a vasculitis that affects medium it can be suspected clinically, the gold standard for diagnosis
and large caliber vessels (the aorta and its proximal branches), continues to be the temporal artery biopsy; however, it can
predominantly in people over 50 years of age. Its prevalence is be negative in a significant percentage of patients and is not
low and has been estimated at 2.2 per 10,000 patients in the exempt from complications, which makes it necessary to use
United Kingdom,1 with an incidence in populations of Scan- non-invasive diagnostic methods, such as Doppler ultrasound
dinavian descent of up to 19.8 per 100,000.2 These data have of the temporal arteries.7
not been established in Colombia. In the study conducted by
Ochoa et al. in 2009, of 857 cases of vasculitis reported in
Colombia, only 9 corresponded to GCA.3 The inflammation Material and methods
of the vessels wall leads to a thickening of the intima that
causes the reduction of blood flow with secondary ischemic A bibliographic search was systematized to answer, in
phenomena. a qualitative manner, the question about the usefulness
Histologically, it is characterized by the vascular infiltration of ultrasonography and Doppler in the diagnosis of GCA
of CD4+ T cells and macrophages that subsequently fuse to (Annex 1, Supplementary data). The search was conducted in
form the so-called giant cells.4 PubMed, Embase, Web of Science, Google Scholar, Cochrane
Clinically, 2 phenotypes of presentation have been defined and Epistemonikos, using the search terms defined for
according to the affected arteries and the territory irrigated each database and the different Boolean connectors (Annex
by these vessels: a cranial phenotype, in which the charac- 2, Supplementary data). It was limited to English and
teristic symptoms are headache, especially in the temporal Spanish. The included studies should ideally compare ultra-
area, hypersensitivity of the scalp and jaw claudication. This sonography with the reference standard (temporal artery
subtype is the one that most frequently includes, as a seri- biopsy), or assess the operational characteristics of this diag-
ous and catastrophic manifestation, visual loss secondary to nostic test compared to other diagnostic strategies such
ischemic optic neuritis (anterior or posterior) of arteritic type as nuclear magnetic resonance image, or clinical crite-
and usually affects the older age group. On the other hand, the ria.
extracranial phenotype, also called large-vessel GCA, affects The defined period was from January 1, 1990, until March
the aorta and its main proximal branches, especially of the 31, 2020. The search was conducted by 4 of the authors in an
upper extremities; it occurs with constitutional symptoms, autonomous manner (DJA, AV, CHMV, MPT). In case of dis-
fever, claudication of the upper limbs and aortitis, and patients agreements, these were settled by one of the authors (DJA)
are usually younger than those with the intracranial pheno- with thematic and methodological expertise (Fig. 1).8 For the
type.5 evaluation of the risk of bias of the publications, the 6 domains
Polymyalgia rheumatica is closely related to GCA, it is asso- of the Cochrane Collaboration tool for assessing the risk of bias
ciated in up to 40%–50% of patients, while GCA can be found were considered (Table 1).
 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212 205

Table 1 – Data extraction on the performance of temporal arteries ultrasound.

Author and year Type of study Comparator Performance Risk of Reference
bias
14
Schmidt et al., 1997 Prospective, age- TAB Halo: S 73% E 100% stenosis or occlusion: Medium
and sex-matched S 80% E 93%
controls
(rheumatoid
arthritis)
Halo, stenosis or occlusion: S 93% E 93%
26
Nesher et al., 2002 Prospective, TAB or clinical Halo vs. diagnostic criteria: S 86% E 78% Medium
uncontrolled diagnosis PPV 50% NPV 96% Halo vs. TAB: S 78% E
61% PPV 44% NPV 88%
22
Karassa et al., 2005 SLR + meta-analysis TAB or diagnostic Halo vs. TAB: S 69% E 82% Medium
criteria
Stenosis or occlusion vs. TAB: S 68% E 77%
Halo, stenosis or occlusion vs. TAB: S 88%
E 78% Halo vs. diagnostic criteria: S 55% E
94%
Stenosis or occlusion vs. diagnostic
criteria: S 66% E 95%
Halo, stenosis or occlusion vs. diagnostic
criteria: S 87% E 96%
30
Karahaliou et al., Prospective, TAB or clinical Halo vs. clinical diagnosis or TAB: S 82% E Medium
2006 uncontrolled diagnosis 91% PPV 86% NPV 88%
24
Ball et al., 2010 SLR + meta-analysis TAB or diagnostic Halo vs. TAB: S 75% E 83% Low
criteria
Halo, stenosis or occlusion vs. TAB: S 83%
E 82%
Halo vs. diagnostic criteria: S 69% E 89%
Halo, stenosis or occlusion vs. diagnostic
criteria: S 78% E 88%
Halo, stenosis or occlusion vs. TAB or
diagnostic criteria (without previous use
of steroids): S 75% E 88%
Halo, stenosis or occlusion vs. TAB or
diagnostic criteria (with previous use of
steroids): S 72% E 87%
23
Arida et al., 2010 Literature review + Diagnostic criteria Pooled sensitivity 68% Pooled specificity Medium
pooled analysis 91%
28
Aschwanden et al., Prospective, Diagnostic criteria Halo with or without stenosis: S 55% E Medium
2010 uncontrolled 100% PPV 100% NPV 67%
29
Hauenstein et al., Retrospective, TAB or clinical Halo vs. clinical diagnosis: Medium
2012 uncontrolled diagnosis
Steroids on days 0−1 before US: S 87.5% E
91.7%
Steroids on days 2−4 before US: S 50% E
100%
Steroids > 4 days before US: S 50% E 80%
Halo vs. TAB:
Steroids on days 0−1 before US: S 92.3% E
57.1%
Steroids on days 2−4 before US: S 80% E
83.3%
Steroids > 4 days before US: S 50% E 25%
31
Aschwanden et al., Prospective, Diagnostic criteria Halo and compression sign: S 79% E 100% Medium
2012 uncontrolled PPV 100% NPV 80%
27
Diamantopoulos Prospective, Clinical diagnosis Halo in temporal artery: S 96% E 90% Medium
et al., 2014 uncontrolled
Halo in temporal or axillary artery: S 98%
E 91%
Halo in temporal + axillary + common
carotid arteries: S 100%
32
Aschwanden et al., Prospective, Diagnostic criteria Compression sign: S 79% E 100% Medium
2015 uncontrolled
44
Luqmani et al., 2016 Prospective TAB US: S 54% E 81% Medium
multicenter,
uncontrolled
206 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212

– Table 1 (Continued)
Author and year Type of study Comparator Performance Risk of Reference
bias

Clinical judgment at 2 weeks + US: S 93%

E 77%
25
Duftner et al., 2017 SLR + meta-analysis TAB or clinical Halo vs. clinical diagnosis: S 77% E 96% Low
diagnosis LR+ 19 LR− 0,2
Halo, stenosis or occlusion vs. clinical
diagnosis: S 78% E 89% LR+ 6.9 LR− 0.3
Halo vs. TAB: S 70% E 84% LR+ 4.3 LR− 0.4
Halo or stenosis vs. TAB: S 77% E 91% LR+
8.4 LR− 0.3
Halo, stenosis or occlusion vs. TAB: S 78%
E 91% LR+ 8.3 LR− 0.2

Halo vs. clinical diagnosis: excluding

studies with high risk of bias: S 86% E 95%
LR+ 16 LR− 0.2
Studies without glucocorticoids before
US: S 73% E 82% LR+ 4 LR− 0,3
Studies with high-resolution ultrasound
system: S 87% E 94% LR+ 13.8 LR− 0.1
Well-defined suspected diagnosis: S 89% E
92% LR+ 11.5 LR− 0.1
Longitudinal studies: S 88% E 88% LR+ 7.5
LR− 0.1
21
Schäfer et al., 2017 Prospective, age- Diagnostic criteria Common superficial temporal artery: Low
and sex-matched cut-off of the intima media of 0.42 mm S
controls 100% E 100%
34
Chrysidis et al., 2018 Delphi, 150 images – Interobserver agreement 91%−99%, kappa Low
0.83−0.98

E: specificity; LR: Likelihood ratio; SLR: systematic literature review; S: sensitivity; TAB: temporal artery biopsy; US: temporal artery ultrasound;
NPV: negative predictive value; PPV: positive predictive value.

How is giant cell arteritis diagnosed? Role of the Doppler ultrasound of the temporal
arteries
In 1990, the American College of Rheumatology defined 5
classification criteria for the disease: age over 50 years, new- Since the 1970s, the Doppler ultrasound of the temporal arter-
onset localized headache, tenderness in the temporal artery ies has been used, initially to locate them before the biopsy.13
or decreased temporal artery pulse, erythrocyte sedimenta- In 1997, Schmidt et al. proposed this exam as a diagnostic
tion rate higher than 50 mm/h and suggestive findings in tool14 for this disease since it was a non-invasive, quick and
the temporal artery biopsy: mononuclear infiltrate or gran- inexpensive technique, usually well tolerated, without expo-
ulomatous process with multinucleated giant cells. If 3 of sure to contrast media, which provides information on the
these criteria were met, the patient was classified as hav- presence of edema of the vessel wall and allows a dynamic
ing GCA, with a sensitivity of 93.5% and a specificity of evaluation around its full extension, which entails an advan-
91.2%.9 tage over the biopsy, since it overcomes the inconvenience of
The disease can be suspected with the symptoms described the fact that the affection might be segmental15 ; in addition,
in a patient over 50 years of age with elevated acute phase it allows the evaluation of the superficial temporal artery and
reactants; however, the gold standard for the diagnosis is the other cranial branches, as well as of extracranial vessels such
temporal artery biopsy, despite its variable sensitivity between as the axillary arteries.16
49 and 85% depending on the sample length, the involvement Its main disadvantage is that it is an operator-dependent
of arteries other than the temporal, the segmental nature of technique. The expertise of the operator, especially an ade-
the inflammation and the time of treatment with glucocor- quate learning curve in positive cases, and the optimal quality
ticoids.10,11 The complications from this procedure are not of the ultrasound system influence the performance of the
frequent, but may include arterial or venous bleeding, infec- study. The European League Against Rheumatism (EULAR)
tion of the surgical site, and injury to the auriculotemporal recommends ultrasound of the temporal arteries as the first
nerve or to branches of the facial nerve.12 imaging technique in patients with suspected GCA with
 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212 207

Fig. 1 – PRISMA search flowchart.

predominantly cranial involvement and highlights the impor- ence point to define the origin of the 2 branches. The entire
tance of an optimal ultrasonography assessment performed course of the common temporal artery and its branches should
by a trained specialist and with an appropriate technical be evaluated.
equipment.17 The axillary artery is evaluated with the transducer on the
mid-axillary line and a scan is made following its anatomical
trajectory. The transducer must be applied both in a longi-
Technique
tudinal and a transverse plane. This technique can be used
by rheumatologists, radiologists and specialists in vascular
Ultrasound and Doppler techniques for the assessment of medicine, with the particularity that whoever does it must
temporal and axillary arteries should be performed with a have adequate training in echography and ultrasound, espe-
linear transducer; there are 3 Doppler techniques: Spectral cially of the temporal arteries. The minimum recommended
Doppler, which consists in a curve of velocity in the Y axis and technical characteristics of the equipment are detailed below
time in the X axis, which represents the variation in flow; Color (it should be noted that these specifications have been pro-
Doppler, which, according to the mean flow velocity assigns a posed by consensus of experts),15,17,18
color on a predetermined scale, and that overlaps the B-mode;
finally, the Power Doppler observes the flow intensity, unlike
the previous ones that determine the velocity. The most widely
used Doppler technique for the assessment of the flow of the
Grayscale
temporal arteries is Color Doppler.
With regard to Color Doppler, it should be performed at a Frequency: ≥15 MHz for temporal arteries and between 7–15
frequency of at least 10 MHz with a vascular pre-set. The eval- MHz for extracranial arteries.
uation of the temporal arteries should be done in the supine Focus: it should be on the artery. In general, 5 mm for tem-
position. The first portion of the temporal artery is visualized poral arteries, 20–30 mm for axillary arteries.
in the tragus. The transducer should be in a transverse plane, Depth: in general, 10–20 mm for temporal arteries, 30–40
and later in a longitudinal plane. After completing an ini- mm for axillary arteries.
tial scan with one plane, the transducer is rotated 90 degrees B-mode gain: on average, 35−45 dB.
to evaluate the opposite plane. The level of the bifurcation Linear density: 3.
between the frontal and parietal branches is used as a refer- Dynamic range: 40−60 dB.
208 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212

and a negative predictive value of 96%.26 The sensitivity of

Doppler ultrasound only increases by 2% when the evalu-
ation of the axillary arteries is added to the study of the
temporal arteries27 . Different studies have shown that in
the majority of patients the halo sign is not visible after
2–4 weeks following the start of the therapy with glucocor-
ticoids, because the inflammatory changes disappear and
the sensitivity of the study decreases to 50% from the 4th
day of treatment, so early evaluation with Doppler ultra-
sound is recommended in the first 2 days after starting the
treatment.28–30
2 Temporal artery compressibility sign (Fig. 3): it is the persis-
tence of the hypoechoic halo despite extrinsic compression
of the arterial lumen with the ultrasound transducer («sign
Fig. 2 – Halo sign in Doppler ultrasound. of compressibility»); it is a variant of the halo with a sensi-
tivity of 77%–79% and a specificity of 100%,31,32
3 Stenosis: it occurs when the blood flow velocity is more than
twice the recorded in the immediately anterior area, with
Color Doppler waveforms which demonstrate turbulence and reduction in
velocity behind the area of stenosis16 .
Frequency: Between 7 and 12 MHz for temporal arteries and 4 Occlusion: complete absence of flow. In the study conducted
between 4 and 8 MHz for extracranial arteries. by Schmidt et al. both stenosis and occlusions had a sensi-
Pulse repetition frequency: 2–3.5 kHz for temporal arteries and tivity of 80% and a specificity of 93%16 . The finding of arterial
between 3 and 4 kHz for extratemporal arteries. stenosis or occlusion does not increase the diagnostic per-
Wall filter: the least possible filter. formance of the halo sign25 .
Color box: it should be at an insonation angle ≤ 60◦ .
Color gain: between 2 and 18. In order to standardize and evaluate the performance of
Doppler ultrasound in the diagnosis of large vessel vasculi-
tis, the OMERACT LV-US (Outcome Measures in Rheumatology
Pathological ultrasonographic findings in the Large Vessel Ultrasound) working group was constituted
patient with giant cell arteritis within the framework of the annual congress of the American
College of Rheumatology in Boston in 2014.16 Based on this
4 pathological findings can be documented in the ultrasound strategy, it was published a first study whose objective was to
of the temporal arteries of a patient with GCA16 : define the fundamental ultrasound findings of GCA and eval-
uate its performance according to these findings. 25 experts
1 Halo sign (Fig. 2): it is a homogeneous thickening of the in Doppler ultrasound in CGA participated obtaining a total
wall, hypoechoic (in mode B), with defined edges (it is of 9 definitions on findings consisting in normal appearance,
possible to delimit the tissue adjacent to the vessel), visi- vasculitis and atherosclerosis of cranial and extracranial arter-
ble in the transverse (usually concentric) and longitudinal ies. It was found that halo and compression signs are the key
planes. It reflects the edema of the vessel wall and it is findings on Doppler ultrasound of patients with GCA.
the most relevant finding for this disease. Some studies The definitions of normal temporal and axillary arteries,
defined cut-off points of the diameter of the halo for tempo- halo and compressibility signs showed an interobserver agree-
ral arteries between 0.3 and 1.0 mm and for axillary arteries ment of 91%−99%, with a mean kappa value of 0.83−0.98
between 1.0 and 2.0 mm.19,20 Schafer et al., in a prospective among the expert evaluators.33 In a recent case-control study,
study comparing 40 patients with GCA and healthy con- it was demonstrated an adequate inter-and intra-observer
trols, found that the normal diameter of the intima-media concordance of the compressibility and halo signs among
thickness (IMT) was on average 0.2 mm in the temporal previously trained operators (more than 300 studies), with a
arteries and 0.6 mm in the axillary arteries, and they estab- standardized study protocol of at least 15−20 min per study
lished cut-off points for the IMT of patients with GCA in and using transducers of more than 15 MHz.34
the common superficial temporal arteries of 0.65 mm (sen-
sitivity and specificity of 100%), in the frontal branch of
0.54 mm (sensitivity and specificity of 100%), in the pari-
etal branch of 0.50 mm (sensitivity of 97.2% and specificity False positives of the halo sign on Doppler
of 98.7%), in the facial artery of 0.53 mm (sensitivity of ultrasound
87.5% and specificity of 98.8%) and in the axillary arteries
of 1.7 mm (sensitivity and specificity of 100%).21 Several False positives of the halo sign have been described in
meta-analyses have reported a sensitivity of 68%–77% and other forms of vasculitis (such as polyarteritis nodosa and
a specificity of 83%–96% for the unilateral halo sign and a the anti-neutrophil cytoplasmic antibodies (ANCA)-associated
sensitivity of 43% and a specificity of 100% for the bilat- vasculitis, amyloidosis, neoplasms, infections and atheroscle-
eral halo sign,22–25 with a positive predictive value of 50% rosis.26,35–38 Table 2 summarizes the possible mechanisms
 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212 209

Fig. 3 – Compressibility and halo in the temporal artery.

Table 2 – False positives of the halo sign in Doppler ultrasound.

Disease Proposed mechanism Affected arteries

Atherosclerosis Deposit of material in the vascular wall Axillary, temporal

Increased IMT
ANCA-related vasculitis Cell infiltration and edema Temporal
Skull base osteomyelitis Cell infiltration and edema Temporal
Neurosyphilis Cell infiltration and edema Temporal
T-cell lymphoma Cell infiltration and edema Temporal
Multiple myeloma Deposit of material in the vascular wall Temporal
Amyloidosis Deposit of material in the vascular wall Temporal

ANCA: antineutrophil cytoplasmic antibodies; IMT: intima-media thickness.

implied in the presence of the halo sign in diseases other than

GCA.
Could the temporal artery biopsy be replaced
The foregoing demands that the results of the Doppler
by Doppler ultrasound in the diagnosis of
ultrasound be interpreted in the context of the clinical and
patients with giant cell arteritis?
laboratory findings, and also requires the optimization and
standardization of the imaging protocols in the diagnostic Ultrasound-guided biopsy of the temporal arteries does not
process, as well as the establishment of cut-off points (hope- appear to increase the sensitivity of the histological study for
fully in the different populations) to reduce false positive the diagnosis of the disease. In the presence of a positive halo
rates.18,20,21,39 sign, the probability of having a positive biopsy is equally high,
Both atherosclerosis and CGA increase in frequency with regardless of whether the biopsy is guided by ultrasound or by
age. In atherosclerosis, calcified plaques of hyperechoic clinical evaluation.30 The use of Doppler ultrasound has been
appearance are produced or an increase in the IMT is proposed as a possible diagnostic tool to prescind from the
generated, which is visualized hyperechoic on Doppler ultra- biopsy in selected patients,14,43 although some studies report
sound.40,41 Until now, there is no consensus to properly contradictory results in which Doppler ultrasound does not
differentiate between an inflammatory involvement of the surpass the clinical evaluation39 and it has a better perfor-
vascular wall and atherosclerotic changes, especially when mance when it is negative to rule out the disease, due to its
there is no predominant calcium component. Some authors high negative predictive value.26 However, works published
suggest that the carotid IMT shows a significant correlation after 2010, in which ultrasound systems of higher resolution
with the temporal IMT: A carotid IMT > 0.9 mm was associated were used, demonstrated performances comparable with the
with a temporal IMT > 0.3 mm, simulating a halo sign, so they temporal artery biopsy for the diagnosis of GCA.25
propose a cut-off point of IMT > 0.34 in at least 2 branches of The TABUL study assessed the performance and cost-
the temporal artery to reduce the number of false positives42 . effectiveness of Doppler ultrasound of the axillary and
More studies are needed to validate these findings. temporal arteries compared with temporal artery biopsy in
210 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212

the diagnosis of GCA in 381 patients. The results showed ischemic complications of the disease. In our environment is
a sensitivity of the Doppler ultrasound of 54% and of the essential to foster research and training of the personnel who
temporal artery biopsy of 39%, with specificities of 81% and will be in charge of these patients.
100%, respectively. The interobserver correlation of the sono-
graphers in a post hoc evaluation was comparable with that
of the pathologists (intraclass correlation coefficient: 0.61 vs. Conflict of interest
0.62, respectively).44
The EULAR recommends to evaluate the temporal arteries The authors declare that they have no conflict of interest.
initially; and if the study is negative and clinical suspicion per-
sists, additional vessels such as the axillary arteries and other Appendix A. Supplementary data
cranial or extracranial arteries should be evaluated.17 Patients
with clinical signs of GCA and a positive halo sign on Doppler
Supplementary material related to this article can be
ultrasound could be treated without the need for a tempo-
found, in the online version, at doi:https://doi.org/10.1016/j.
ral artery biopsy, unless there is suspicion of another type of
rcreue.2020.08.002.
vasculitis (such as the ANCA-associated, which would show a
different histopathology). Those who have strong clinical evi-
dence of GCA, but only have stenoses or occlusions, should references
undergo a temporal artery biopsy. Patients with clinical signs
of polymyalgia rheumatica without symptoms of GCA but who
have abnormal findings on Doppler ultrasound (halo, steno- 1. Barraclough K, Mallen CD, Helliwell T, Hider SL, Dasgupta B.
sis or occlusion) should undergo biopsy and receive treatment Diagnosis and management of giant cell arteritis. Br J Gen
Pract. 2012;62:329–30,
with glucocorticoids while the results of the biopsy are known
http://dx.doi.org/10.3399/bjgp12X649313.
in order to avoid visual loss.14 2. Chandran A, Udayakumar P, Crowson C, Warrington K,
The fast-track ultrasound clinics for the early diagnosis of Matteson E. The incidence of giant cell arteritis in Olmsted
GCA were created with the aim of reducing the ischemic com- County, Minnesota, over a 60-year period 1950-2009. Scand J
plications of the disease, especially the permanent visual loss, Rheumatol. 2015;44:215–8,
since it has been demonstrated that the early detection and http://dx.doi.org/10.3109/03009742.2014.982701.
3. Ochoa CD, Ramírez F, Quintana G, Toro C, Cañas C, Osio LF,
initiation of glucocorticoid treatment improves outcomes.45
et al. Epidemiología de las vasculitis primarias en Colombia y
Outpatients with clinical suspicion of GCA undergo rapid clin-
su relación con lo informado para Latinoamérica. Rev Colomb
ical and ultrasound evaluation by trained specialists, and Reumatol. 2009;16:248–63.
glucocorticoid therapy is promptly initiated if indicated. In a 4. Lazarewicz K, Watson P. Giant cell arteritis. BMJ.
reference center which uses this modality of care, the evalu- 2019;365:l1964, http://dx.doi.org/10.1136/bmj.l1964.
ation by a fast-track ultrasound model in patients with CGA 5. Rinden T, Miller E, Nasr R. Giant cell arteritis: an updated
demonstrated a reduction of 88% in the risk of permanent review of an old disease. Cleve Clin J Med. 2019;86:465–72,
http://dx.doi.org/10.3949/ccjm.86a.18103.
visual loss, compared with the conventional model (RR 0.12;
6. Gonzalez-Gay MA, Barros S, Lopez-Diaz MJ, Garcia-Porrua C,
95% CI: 0.01−0.97; P = .01) and the mean difference in days
Sanchez-Andrade A, Llorca J. Giant cell arteritis: disease
of hospitalization between the patients evaluated in a con- patterns of clinical presentation in a series of 240 patients.
ventional manner and the patients evaluated by fast-track Medicine (Baltimore). 2005;84:269–76,
ultrasound was 3 days (3.6 vs. 0.6 days; P < .0005).46 http://dx.doi.org/10.1097/01.md.0000180042.42156.d1.
7. Hayreh SS, Zimmerman B, Kardon RH. Visual improvement
with corticosteroid therapy in giant cell arteritis. Report of a
large study and review of literature. Acta Ophthalmol Scand.
Conclusions
2002;80:355–67,
http://dx.doi.org/10.1034/j.1600-0420.2002.800403.x.
Giant cell arteritis, although infrequent, is an entity that gen- 8. Moher D, Liberati A, Tetzlaff J, Altman DG. Preferred reporting
erates high morbidity. Timely recognition and early treatment items for systematic reviews and meta-analyses: the PRISMA
can prevent serious complications, such as visual loss. The statement. PLoS Med. 2009;6:e1000097.
clinical challenge represented by its diagnosis and the lim- 9. Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP,
Calabrese LH, et al. The American College of Rheumatology
itations of the gold standard have opened the door to new
1990 criteria for the classification of giant cell arteritis.
imaging techniques. Color Doppler is an inexpensive, fast,
Arthritis Rheum. 1990;33:1122–8,
and easily available tool with good diagnostic performance. http://dx.doi.org/10.1002/art.1780330810.
However, it is essential to have trained personnel, appropriate 10. Horton B, Magath T, Brown G. An undescribed form of
ultrasound equipment and to use the adequate programming. arteritis of the temporal vessels. Mayo Clin Proc. 1932;7:700–1.
Doppler findings, such as the halo sign, the lack of com- 11. Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, Gonzalez-Louzao
pressibility of the temporal artery, the stenosis and occlusion C, Rodriguez-Ledo P. Biopsy-negative giant cell arteritis:
clinical spectrum and predictive factors for positive temporal
are powerful evidence for the diagnosis of GCA. Among these
artery biopsy. Semin Arthritis Rheum. 2001;30:249–56,
findings, the most studied sign is the halo, which has a good
http://dx.doi.org/10.1053/sarh.2001.16650.
diagnostic performance. However, this sign has findings that 12. Albertini JG, Ramsey ML, Marks VJ. Temporal artery biopsy in
can lead to false positives and that must be taken into account a dermatologic surgery practice. Dermatologic Surg.
in a differential diagnosis. Lastly, the fast-track clinics sup- 1999;25:501–8,
ported by ultrasound and Doppler have managed to reduce the http://dx.doi.org/10.1046/j.1524-4725.1999.08296.x.
 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212 211

13. Kelley JS. Doppler ultrasound flow detector used in temporal giant cell arteritis determined by duplex sonography of 2x11
artery biopsy. Arch Ophthalmol. 1978;96:845–6, arterial regions. Ann Rheum Dis. 2010;69:1356–9,
http://dx.doi.org/10.1001/archopht.1978.03910050451010. http://dx.doi.org/10.1136/ard.2009.122135.
14. Schmidt WA, Kraft HE, Vorpahl K, Völker L, Gromnica-Ihle EJ. 29. Hauenstein C, Reinhard M, Geiger J, Markl M, Hetzel A, Treszl
Color duplex ultrasonography in the diagnosis of temporal A, et al. Effects of early corticosteroid treatment on magnetic
arteritis. N Engl J Med. 1997;337:1336–42, resonance imaging and ultrasonography findings in giant cell
http://dx.doi.org/10.1056/NEJM199711063371902. arteritis. Rheumatology. 2012;51:1999–2003,
15. Terslev L, Diamantopoulos AP, Døhn UM, Schmidt WA, http://dx.doi.org/10.1093/rheumatology/kes153.
Torp-Pedersen S. Settings and artefacts relevant for Doppler 30. Karahaliou M, Vaiopoulos G, Papaspyrou S, Kanakis MA,
ultrasound in large vessel vasculitis. Arthritis Res Ther. Revenas K, Sfikakis PP. Colour duplex sonography of temporal
2017;19:167, http://dx.doi.org/10.1186/s13075-017-1374-1. arteries before decision for biopsy: a prospective study in 55
16. Schmidt WA. Ultrasound in the diagnosis and management patients with suspected giant cell arteritis. Arthritis Res Ther.
of giant cell arteritis. Rheumatology. 2018;57 Supl. 2:ii22–31, 2006;8:R116, http://dx.doi.org/10.1186/ar2003.
http://dx.doi.org/10.1093/rheumatology/kex461. 31. Aschwanden M, Daikeler T, Kesten F, Baldi T, Benz D, Tyndall
17. Dejaco C, Ramiro S, Duftner C, Besson FL, Bley TA, Blockmans A, et al. Temporal artery compression sign - A novel
D, et al. EULAR recommendations for the use of imaging in ultrasound finding for the diagnosis of giant cell arteritis.
large vessel vasculitis in clinical practice. Ann Rheum Dis. Ultraschall der Medizin - Eur J Ultrasound. 2012;34:47–50,
2018;77:636–43, http://dx.doi.org/10.1055/s-0032-1312821.
http://dx.doi.org/10.1136/annrheumdis-2017-212649. 32. Aschwanden M, Imfeld S, Staub D, Baldi T, Walker UA, Berger
18. Monti S, Floris A, Ponte C, Schmidt WA, Diamantopoulos AP, CT, et al. The ultrasound compression sign to diagnose
Pereira C, et al. The use of ultrasound to assess giant cell temporal giant cell arteritis shows an excellent interobserver
arteritis: review of the current evidence and practical guide agreement. Clin Exp Rheumatol. 2015;33 2 Suppl 89:
for the rheumatologist. Rheumatology. 2018;57:227–35, S-113–115.
http://dx.doi.org/10.1093/rheumatology/kex173. 33. Chrysidis S, Duftner C, Dejaco C, Schäfer VS, Ramiro S,
19. De Miguel E, Roxo A, Castillo C, Peiteado D, Villalba A, Carrara G, et al. Definitions and reliability assessment of
Martín-Mola E. The utility and sensitivity of colour Doppler elementary ultrasound lesions in giant cell arteritis: a study
ultrasound in monitoring changes in giant cell arteritis. Clin from the OMERACT Large Vessel Vasculitis Ultrasound
Exp Rheumatol. 2012;30 1 Suppl 70:S34–8. Working Group. RMD Open. 2018;4:e000598,
20. Czihal M, Schröttle A, Baustel K, Lottspeich C, Dechant C, http://dx.doi.org/10.1136/rmdopen-2017-000598.
Treitl K-M, et al. B-mode sonography wall thickness 34. Schäfer VS, Chrysidis S, Dejaco C, Duftner C, Iagnocco A,
assessment of the temporal and axillary arteries for the Bruyn GA, et al. Assessing vasculitis in giant cell arteritis by
diagnosis of giant cell arteritis: a cohort study. Clin Exp ultrasound: results of OMERACT patient-based reliability
Rheumatol. 2017;35 Supl. 1:128–33. exercises. J Rheumatol. 2018;45:1289–95,
21. Schäfer VS, Juche A, Ramiro S, Krause A, Schmidt WA. http://dx.doi.org/10.3899/jrheum.171428.
Ultrasound cut-off values for intima-media thickness of 35. Touboul P-J, Hennerici MG, Meairs S, Adams H, Amarenco P,
temporal, facial and axillary arteries in giant cell arteritis. Bornstein N, et al. Mannheim carotid intima-media thickness
Rheumatology. 2017;56:1479–83, and plaque consensus (2004-2006-2011). Cerebrovasc Dis.
http://dx.doi.org/10.1093/rheumatology/kex143. 2012;34:290–6, http://dx.doi.org/10.1159/000343145.
22. Karassa FB, Matsagas MI, Schmidt WA, Ioannidis JPA. 36. Argyropoulou OD, Protogerou AD, Sfikakis PP. Accelerated
Meta-analysis: test performance of ultrasonography for. Ann atheromatosis and arteriosclerosis in primary systemic
Intern Med. 2005;142:359–69, vasculitides. Curr Opin Rheumatol. 2018;30:36–43,
http://dx.doi.org/10.7326/0003-4819-142-5-200503010-00011. http://dx.doi.org/10.1097/BOR.0000000000000453.
23. Arida A, Kyprianou M, Kanakis M, Sfikakis PP. The diagnostic 37. Molina Collada J, Ruíz Bravo-Burguillos E, Monjo I, Bonilla G,
value of ultrasonography-derived edema of the temporal Fernández E, Balsa A, et al. Positive ultrasound halo sign of
artery wall in giant cell arteritis: a second meta-analysis. BMC temporal arteries due to amyloidosis. Rheumatology (Oxford).
Musculoskelet Disord. 2010;11:44, 2019:582067–9,
http://dx.doi.org/10.1186/1471-2474-11-44. http://dx.doi.org/10.1093/rheumatology/kez182.
24. Ball EL, Walsh SR, Tang TY, Gohil R, Clarke JMF. Role of 38. Fernández-Fernández E, Monjo-Henry I, Bonilla G, Plasencia
ultrasonography in the diagnosis of temporal arteritis. Br J C, Miranda-Carús M-E, Balsa A, et al. False positives in the
Surg. 2010;97:1765–71, http://dx.doi.org/10.1002/bjs.7252. ultrasound diagnosis of giant cell arteritis: some diseases can
25. Duftner C, Dejaco C, Sepriano A, Falzon L, Schmidt WA, also show the halo sign. Rheumatology (Oxford).
Ramiro S. Imaging in diagnosis, outcome prediction and 2020;59:2443–7,
monitoring of large vessel vasculitis: a systematic literature http://dx.doi.org/10.1093/rheumatology/kez641.
review and meta-analysis informing the EULAR 39. Salvarani C, Silingardi M, Ghirarduzzi A, Lo Scocco G,
recommendations. RMD Open. 2018;4:e000612, Macchioni P, Bajocchi G, et al. Is duplex ultrasonography
http://dx.doi.org/10.1136/rmdopen-2017-000612. useful for the diagnosis of giant-cell arteritis? Ann Intern
26. Nesher G, Shemesh D, Mates M, Sonnenblick M, Abramowitz Med. 2002;13:232,
HB. The predictive value of the halo sign in color Doppler http://dx.doi.org/10.7326/0003-4819-137-4-200208200-00006.
ultrasonography of the temporal arteries for diagnosing giant 40. Laclaustra M, Casasnovas JA, Fernández-Ortiz A, Fuster V,
cell arteritis. J Rheumatol. 2002;29:1224–6. León-Latre M, Jiménez-Borreguero LJ, et al. Femoral and
27. Diamantopoulos AP, Haugeberg G, Hetland H, Soldal DM, Bie carotid subclinical atherosclerosis association with risk
R, Myklebust G. Diagnostic value of color Doppler factors and coronary calcium: the AWHS Study. J Am Coll
ultrasonography of temporal arteries and large vessels in Cardiol. 2016;67:1263–74,
giant cell arteritis: a consecutive case series. Arthritis Care http://dx.doi.org/10.1016/j.jacc.2015.12.056.
Res (Hoboken). 2014;66:113–9, 41. Milchert M, Fischer K, Flicinski J, Przepiera-Bedzak H, Brzosko
http://dx.doi.org/10.1002/acr.22178. M. Arteriosclerosis or vasculitis? Color duplex sonography in
28. Aschwanden M, Kesten F, Stern M, Thalhammer C, Walker giant cell arteritis. J Rheumatol. 2012;39:1898–9,
UA, Tyndall A, et al. Vascular involvement in patients with http://dx.doi.org/10.3899/jrheum.120317.
212 r e v c o l o m b r e u m a t o l . 2 0 2 1;2 8(3):203–212

42. De Miguel E, Beltran LM, Monjo I, Deodati F, Schmidt WA, 45. Raza K, Saber TP, Kvien TK, Tak PP, Gerlag DM. Timing the
Garcia-Puig J. Atherosclerosis as a potential pitfall in the therapeutic window of opportunity in early rheumatoid
diagnosis of giant cell arteritis. Rheumatology (Oxford). arthritis: proposal for definitions of disease duration in
2018;57(2):318–21, clinical trials. Ann Rheum Dis. 2012;71:1921–3,
http://dx.doi.org/10.1093/rheumatology/kex381. http://dx.doi.org/10.1136/annrheumdis-2012-201893.
43. Pfadenhauer K, Weber H. Duplex sonography of the temporal 46. Diamantopoulos AP, Haugeberg G, Lindland A, Myklebust G.
and occipital artery in the diagnosis of temporal arteritis. A The fast-track ultrasound clinic for early diagnosis of giant
prospective study. J Rheumatol. 2003;30:2177–81. cell arteritis significantly reduces permanent visual
44. Luqmani R, Lee E, Singh S, Gillett M, Schmidt WA, Bradburn impairment: towards a more effective strategy to improve
M, et al. The role of ultrasound compared to biopsy of clinical outcome in giant cell arteritis? Rheumatology
temporal arteries in the diagnosis and treatment of giant cell (Oxford). 2016;55:66–70,
arteritis (TABUL): a diagnostic accuracy and http://dx.doi.org/10.1093/rheumatology/kev289.
cost-effectiveness study. Health Technol Assess (Rockv).
2016;20:1–238, http://dx.doi.org/10.3310/hta20900.