5 páginasLa arteritis de células gigantes es una enfermedad autoinmune que afecta vasos de mediano y gran calibre, creando depósitos de cúmulos inflamatorios en la pared arterial. Es la forma más común de vasculitis de grandes vasos, pero dada la variabilidad en la eficacia de la biopsia y de otras estrategias diagnósticas empleadas, su diagnóstico es complejo. Presentamos el caso de una paciente de 69 años que presentó déficit neurológico y aumento de la sensibilidad bilateral en la región temporal, asociado a una cefalea intensa. La evaluación diagnóstica reveló calcificación de las arterias temporal superficial, vertebral y oftálmica, así como hallazgos sugestivos en la ecografía Doppler, como el signo del halo, que apuntan a arteritis temporal superficial, aunque no descartan la posibilidad de que dichas calcificaciones sean compatibles con aterosclerosis en una paciente con enfermedad renal crónica avanzada, lo que se ha descrito como causa de falsos positivos. Es importante conocer las principales diferencias entre ambos diagnósticos, dada la amplia gama de posibilidades diagnósticas por imagen que pueden evitar la necesidad de una biopsia

Bastidas, Nicolás

Hernández, Michel

Rincón, Oscar

Rodríguez, Laura C

Radiology Case Reports

English

PubMed

Intellectum (Universidad de La Sabana)

R a d i o l o g y  C a s e  R e p o r t s  1 7  ( 2 0 2 2 )  3 1 9 1 – 3 1 9 5  Available online at www.sciencedirect.com journal homepage: www.elsevier.com/locate/radcr Case Report Temporal superficial arteritis as differential diagnosis in patients with atherosclerotic changes due to advanced chronic renal disease: case report and review of the literature  Michel Hernández, MD, ESP, SPE a , Laura C. Rodríguez, MD b , Nicolás Bastidas, EST b , ∗, Oscar Rincón, MD, SPE a a Hospital Universitario de la Samaritana, Bogota, Colombia b Universidad de la Sabana, Chia, Colombia a r t i c l e i n f o Article history: Received 30 May 2022 Revised 2 June 2022 Accepted 5 June 2022 Available online 28 June 2022 Keywords: Giant cell Vasculitis Temporal artery Calcification Chronic renal disease a b s t r a c t Giant cell arteritis is an autoimmune disease that affects medium and large caliber ves- sels, creating deposits of inflammatory clusters on the arterial wall. It is the most common form of large vessel vasculitis, but given the variability of biopsy efficiency and of other diagnostic strategies employed, the diagnosis of this disease is challenging. We report the case of a 69-year-old female patient who presented with neurological deficit and increased bilateral sensation in the temporal region associated with excruciating headache. Workup revealed calcification of the superficial temporal, vertebral and ophthalmic arteries, as well as suggestive findings on Doppler ultrasound such as the halo sign, pointing to superficial temporal arteritis though not excluding the possibility of those calcifications being consis- tent with atherosclerosis in a patient with advanced chronic renal disease, which has been reported as giving rise to false-positive results. Knowledge of the main differences between the 2 diagnoses is important, given the wide range of diagnostic imaging possibilities which can avoid the need for biopsy. © 2022 The Authors. Published by Elsevier Inc. on behalf of University of Washington. This is an open access article under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ )        Introduction Giant cell arteritis (GCA) is an inflammatory vasculitis affect-ing medium and large vessels and it is the most frequent formAbbreviations: GCA, GIANT CELL ARTERITIS.  Competing Interests: All authors declare no conflict of interest. ∗ Corresponding author at: CRA 8 # 0-29, Universidad de la Sabana, CE-mail address: nicobasgu@hotmail.com (N. Bastidas). https://doi.org/10.1016/j.radcr.2022.06.015 1930-0433/© 2022 The Authors. Published by Elsevier Inc. on behalf of UCC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4hia, Colombia. of vasculitis that occurs in the elderly. It is characterized forbeing systemic and of granulomatous type and is more fre-quent among women, with a median age of onset of 75 years[1] . It must be suspected in the presence of clinical signs suchas headache, amaurosis fugax, transient ischemic attacks orniversity of Washington. This is an open access article under the .0/ ) 3192 R a d i o l o g y  C a s e  R e p o r t s  1 7  ( 2 0 2 2 )  3 1 9 1 – 3 1 9 5  Fig. 1 – Plain cranial CT scan. (A) Calcification of temporal arteries and of the right ophthalmic artery. (B) Bilateral calcification of the vertebral arteries.                                                                  stroke, the latter occurring as early symptoms of the disease,73% at the time of diagnosis, and 27% within the first 2 weeks[2] . It is associated with radiological signs such as temporalartery (TA) calcifications, the halo sign on Doppler ultrasoundand the absence of arterial compressibility, the most frequentbeing the latter 2 [3] . We report the case of patient who pre-sented with neurological deficit and was found to have bilat-eral calcifications of the superficial temporal artery, the oph-thalmic artery and the vertebral arteries, as well as the halosign on superficial temporal artery Doppler. Case description A 69-year-old female patient attended a tertiary level hospi-tal due to surgical site infection one month after supracondy-lar amputation of the right femur and left transtibial ampu-tation secondary to bilateral peripheral arterial occlusive dis-ease. The patient also presented on admission, signs of neu-rological focalization in the form of dysarthria, facial palsyand left hemiparesis, predominantly of the left upper limb andnew onset biparietal throbbing headache of medium intensity.Her medical history included chronic renal disease needingrenal dialysis every other day, diabetes mellitus type II, arte-rial hypertension, heart failure with preserved left ventricularejection fraction, acute myocardial infarction without coro-nary disease, and cor pulmonale. Plain computed tomogra-phy (CT) imaging of the brain showed an ischemic cerebrovas-cular event in the right posterior cerebral artery territory inthe right occipital region, NIHSS score of 3, with no indicationfor thrombolysis. Dual antiplatelet therapy was initiated be-fore referring the patient to our institution for comprehensivemanagement. During the review by systems on admission the patient re-ported dyspnea with mild exertion, recovery of her strengthin the left side of the body. She did not report visual acuity ab-normalities, mandibular claudication or constitutional symp-toms. On initial physical examination, vital signs were nor-mal, with mildly diminished temporal pulses and increasedbilateral tenderness on palpation over the temporal region,symmetrical chest with evidence of systolic murmur, no stri-dor on lung auscultation, and normal abdominal examination;left lower limb with transtibial amputation stump and rightlower limb with supracondylar amputation stump, both withsingle suture margin approximation, in the process of heal-ing. On neurological examination, the patient was alert, fo-cused, oriented in 3 spheres, with mild dysarthria, could re-peat and understand, and had preserved remote and episodicmemory. Cranial nerves with normal reactive isocoric pupil,preserved ocular movements, facial symmetry and preservedsensation. Strength 5/5 in right upper limb and 2/5 in leftupper limb. Osteotendinous reflexes ++ / ++ bilateral flexionstrength, no cerebellar signs, normal gait and no dissymme-try or meningeal signs. On admission to our institution, acute phase reactantswere high, with elevated erythrocyte sedimentation rate(ESR) (65 mm/h); a follow-up plain cranial CT scan showedperiventricular hypodensity possibly due to small vessel hy-poxic/ischemic attack, an 8 mm hypodensity of the cortical-subcortical junction in the frontal region in the middle cere-bral artery territory, and poorly defined acute phase hypoden-sity of ischemic type in the right occipital region, in the terri-tory of the right posterior cerebral artery. Other relevant find-ings included the presence of calcifications of the vertebralarteries, of critical grade in the left vertebral artery, as wellas multiple bilateral calcifications of the superficial temporalartery, involvement and calcification of the ophthalmic arter-ies, suggesting temporal arteritis as the primary cause, versusatherosclerotic changes secondary to chronic renal disease. On follow-up, the patient refused to undergo any inva-sive intervention, precluding the performance of the super-ficial temporal artery biopsy. However, a Doppler ultrasoundof the superficial temporal artery was performed and showedR a d i o l o g y  C a s e  R e p o r t s  1 7  ( 2 0 2 2 )  3 1 9 1 – 3 1 9 5  3193 Fig. 2 – Doppler ultrasound in where it seems a temporal artery wall thickened.                          Table 1 – Clinicopathological criteria from the American College of Rheumatology: 3 out of 5 criteria are necessary to suspect medium vessel vasculitis [4] 1. Age of onset of the disease of 50 years or more 2. New onset headache or location type 3. Tenderness on palpation of the temporal artery or reduced pulse not related to cervical arterial atherosclerosis 4. Erythrocyte sedimentation rate 50 mm/H or greater 5. Positive temporal artery biopsy                   characteristic findings such as thickening of the externalcarotid artery wall and increased resistance in the temporalarteries. Discussion Temporal arteritis, also known as giant cell arteritis, is a formof vasculitis affecting medium and large caliber blood vessels.It can be intra or extracranial, depending on the branches in-volved, but has greater predilection for the temporal and ver-tebral arteries, hence the associated clinical manifestations[5] . It occurs more frequently in white women (2:1 F:M ra-tio) and in individuals over 50 years of age. It is the mostcommon systemic vasculitis of the granulomatous type withchronic presentation; the annual incidence varies between 1.6and 32.8 cases × 100,00 inhabitants in Europe, USA, Israel, andOceania; epidemiological data for Colombia are nonavailable[4] . The triggering agent for this autoimmune disease occur-ring in patients with predisposing factors and HLA class IIpolymorphisms is unknown [8] . Histologically, there is vascu-lar infiltration of CD4 + T cells and macrophages that fuse toform “giant cells” that are deposited on the vessel wall, leadingto arterial intimal hyperplasia and thickening, reduced bloodflow due to stenosis, and arterial occlusion, and giving rise tothe typical symptomatology of this disease [ 2 ,4 ]. The main clinical manifestations have been divided into 2presentation patterns, depending on the affected vessel. Themost frequent with severe manifestations and more preva-lent in the elderly population is the cranial presentation char-acterized by headache, predominantly temporal in 70%-80%,with scalp hypersensitivity and mandibular claudication; itcan even trigger visual loss secondary to ischemic optic neuri-tis. On the other hand, the extracranial phenotype, also calledgiant cell arteritis, affects younger patients and involves theaorta and its proximal branches, the main associated symp-toms being fever, upper limb claudication, aortitis, and consti-tutional symptoms such as weight loss, asthenia and myalgia[ 4 ,6 ]. In 1990, the American College of Rheumatology establishedclinical criteria to help with the classification and suspicion ofthis condition. These criteria are still valid and consist of 4 ofthe cranial symptoms and one temporal artery biopsy and thedisease is suspected if 3 of these are present ( Table 1 ) [5] . The test of choice for the definitive diagnosis of this con-dition is still a subject of debate. Superficial temporal arterybiopsy is found throughout the literature as the gold standardfor confirming the diagnosis. However, its sensitivity variesbetween 39% and 91% given that involvement is segmentaland prior glucocorticoid treatment can result in a high num-ber of false negative results [7] . As part of the search for3194 R a d i o l o g y  C a s e  R e p o r t s  1 7  ( 2 0 2 2 )  3 1 9 1 – 3 1 9 5  Table 2 – Radiological characteristics of temporal superficial arteritis on Doppler ultrasound, the pathognomonic finding is the halo sign [7] Signs Characteristics Halo Eccentric hypoechoic circumferential ring around the vessel ( Fig. 2 ) [7] . Thickness greater than 1.5 mm, highly predictive of GCA [5] . It is the most specific sign. Vascular occlusion Secondary to vessel wall edema. Aliasing is seen due to focal velocity increase with low-resistance beyond the stenosis [3] . Stenosis Peak systolic velocity (PSV) at the site of the TA stenosis is 2 or more times greater than PSV distal or proximal to the stenosis [3] . Arterial compression Persistence of the halo with compression [3] ( Fig. 3 ). Fig. 3 – Persistent TA hypoechoic halo despite compression made by echographic transducer.                              new, less invasive diagnostic options with high sensitivity, theEuropean League Against Rheumatism, in its latest release,recommends Doppler ultrasound as the first imaging studyof choice when temporal arteritis is suspected, avoiding theneed for biopsy [5] . Doppler has shown to be a very useful diagnostic test be-cause of accessibility, noninvasiveness and low cost, althoughit is operator-dependent. The ultrasound assessment consistsof examining the temporal artery and its branches, the exter-nal carotid artery and the axillary artery mainly, but it can alsoinclude the occipital artery or the facial arteries [8] . The mostcommon signs are the presence of a hypoechoic halo, non-compressible vessel, stenosis and calcification ( Table 2 ). CTand magnetic resonance imaging also help as part of the di-agnostic workup, showing calcifications of the temporal, oph-thalmic and vertebral arteries ( Fig. 1 ), taking into considera-tion the main differential diagnoses, namely, atheromatosisand changes due to chronic renal disease. In 2014, Diamantopoulos et al., in a prospective, noncon-trolled cohort study, were able to determine that signs likethe temporal artery halo had a sensitivity of 96% and a speci-ficity of 90% for diagnosis; the halo in the temporal or the ax-illary artery, a sensitivity of 98% and a specificity of 91%, andthe halo in the temporal + axillary + common carotid arter-ies had a sensitivity of 100%. On the other hand, Aschwan-den et al., in another noncontrolled prospective cohort studyin 2015, found that the noncompressibility sign had a sensi-tivity of 79% and a specificity of 100%. This shows that theuse of imaging-based diagnosis is supported in the literature,although the interpretation and any potential limitations ofDoppler ultrasound depend on the technique and the exper-tise of the radiologist in identifying the findings [4] . R a d i o l o g y  C a s e  R e p o r t s  1 7  ( 2 0 2 2 )  3 1 9 1 – 3 1 9 5  3195                               Moreover, there is the question of whether imaging find-ings may be consistent with atherosclerotic changes in a pa-tient with end-stage chronic renal disease, given that calcifica-tion of medium caliber vessels has also been described, creat-ing false-positive results. In a retrospective study, Anwar et al.found that 9.9% of a population of 453 patients with end-stagechronic renal disease showed TA calcification, with calcifica-tion of the intima or the media [9] . The objective of this case report is to propose the possi-bility of diagnosing temporal arteritis without the need forbiopsy, considering that in the context of our patient, clinicaland imaging findings led to the suspicion of this disease con-dition based on the finding on cranial CT of bilateral calcifica-tions of the superficial temporal artery as well as of the oph-thalmic and vertebral arteries. The additional use of Dopplerultrasound increases the odds of diagnosing the disease basedon the presence of the hypoechoic halo and the compressibil-ity signs described above. In the case of this patient who refused temporal arterybiopsy, based on the epidemiological characteristics, the re-ported symptoms, the physical examination, elevated acutephase reactants and the signs seen on cranial CT and Dopplerultrasound, the most probable diagnosis is temporal arteri-tis. Other potential etiologies such as calcifications found inchronic renal disease cannot be ruled out. However, so farthere is no consensus to allow the appropriate distinction be-tween vessel wall inflammation and atherosclerotic changes[4] . Patient consent The tomography images such as the Doppler of the superficialtemporal artery and the clinical history have been authorizedby informed consent by both the patient and her daughter.Formal consent form available upon request. R E F E R E N C E S  [1] Muhammad A , Amir R , Peterfy BK . Arteritis de la temporal. StatPearls; 2021 .[2] González Manrique G , Baldovino Chiquillo A , Claros Ortiz K . Ataque isquémico transitorio como manifestación inicial de arteritis de células gigantes: reporte de caso. Acta Neurol Colomb 2020;36(3):190–5 .[3] Oliveros Ordás DP, Ibnoulkhatib SM, Fontanilla Echeveste PT, Lara Huéscar DL, Rubio Sánchez DC, San Miguel Espinosa DJ. Ecografía Doppler en la arteritis de la temporal. Seram 2021(1):11–14. Recuperado a partir de https://piper. espacio-seram.com/index.php/seram/article/view/4693 .[4] Urrego-Callejas T , Jaramillo-Arroyave D , Vanegas-García A ,Muñoz-Vahos C , Plaza Tenorio M . Ultrasonido Doppler de arterias temporales en pacientes con arteritis de células gigantes: estado del arte y revisión sistemática de la literatura. Rev Colomb Reumatol 2020;28(3):203–12 .[5] Wolff C V, Paolinelli G P, Guevara HDLD. Arteritis de células gigantes: actualización y proposición de un algoritmo de estudio. Rev Med Chil [Internet] 2020;148(11):1619–29 Disponible en. doi: 10.4067/s0034-98872020001101619 .[6] Winkler A , True D . Giant cell arteritis: 2018 review. Mo Med 2018;115(5):468–70 .[7] Zou Q, Ma S, Zhou X. Retraction note: ultrasound versus temporal artery biopsy in patients with giant cell arteritis: a prospective cohort study. BMC Med Imaging [Internet] 2020;20(1):54 Disponible en. doi: 10.1186/s12880- 020- 00454- 7 .[8] Jese R , Rotar Ž, Tomši ̌c M , Ho ̌cevar A . OP0133 colour Doppler ultrasonography of facial and occipital arteries in patients with giant cell arteritis: the frequency of involvement and the role of their assessment in daily practice: a prospective study. en: oral presentations. BMJ Publishing Group Ltd and European League Against Rheumatism; 2017 .[9] Anwar Z, Zan E, Carone M, Ozturk A, Sozio SM, Yousem DM. Superficial temporal artery calcification in patients with end-stage renal disease: association with vascular risk factorsand ischemic cerebrovascular disease. Indian J Radiol Imaging[Internet] 2011;21(3):215–20 Disponible en. doi: 10.4103/0971-3026.85371 .

Temporal superficial arteritis as differential diagnosis in patients with atherosclerotic changes due to advanced chronic renal disease: case report and review of the literature

Giant cell arteritis is an autoimmune disease that affects medium and large caliber vessels, creating deposits of inflammatory clusters on the arterial wall. It is the most common form of large vessel vasculitis, but given the variability of biopsy efficiency and of other diagnostic strategies employed, the diagnosis of this disease is challenging. We report the case of a 69-year-old female patient who presented with neurological deficit and increased bilateral sensation in the temporal region associated with excruciating headache. Workup revealed calcification of the superficial temporal, vertebral and ophthalmic arteries, as well as suggestive findings on Doppler ultrasound such as the halo sign, pointing to superficial temporal arteritis though not excluding the possibility of those calcifications being consistent with atherosclerosis in a patient with advanced chronic renal disease, which has been reported as giving rise to false-positive results. Knowledge of the main differences between the 2 diagnoses is important, given the wide range of diagnostic imaging possibilities which can avoid the need for biopsy

Rodríguez, Laura C.

PubMed Central

Temporal superficial arteritis as differential diagnosis in patients with atherosclerotic changes due to advanced chronic renal disease: case report and review of the literature()

https://intellectum.unisabana.edu.co/bitstream/10818/64318/1/1-s2.0-S1930043322004551-main.pdf

Temporal superficial arteritis as differential diagnosis in patients with atherosclerotic changes due to advanced chronic renal disease: case report and review of the literature

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