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Large vessel vasculitides (LVV) comprise a group of inflammatory disorders that involve the large arteries, such as the aorta and its primary branches. The cause of LVV is often rheumatologic and includes giant cell arteritis and Takayasu arteritis. Giant cell arteritis is the most common form of LVV affecting people >50 years of age with a slight female predominance. Takayasu arteritis is more frequently seen in younger populations and is significantly more common in women. Prompt identification of LVV is crucial as it can lead to debilitating complications if left untreated, including blindness in the case of giant cell arteritis and large artery stenosis and aneurysms in the case of all forms of LVV. Noninvasive imaging methods have greatly changed the approach to managing LVV. Today, imaging (with ultrasound, magnetic resonance imaging, computed tomography, and positron emission tomography) is routinely used in the diagnosis of LVV. In patients with giant cell arteritis, imaging often spares the use of invasive procedures such as temporal artery biopsy. In addition, vascular imaging is also crucial for longitudinal surveillance of arterial damage. Finally, imaging is currently being studied for its role in assessing treatment response and ongoing disease activity and its potential value in determining the presence of vascular wall remodeling (eg, scarring). This review explores the current uses of noninvasive vascular imaging in LVV., Competing Interests: Dr Tawakol receives research support (to his institution) from Lung Biotechnologies and consulting fees from Cunningham Bounds LLC for unrelated work and is supported by National Institutes of Health (NIH) R01HL152957, R33HL141047, R01HL1495, P01HL131478, R01AR077187, and R01HL16433, and the International Atomic Energy Agency (IAEA) for unrelated work. Dr Weber receives consulting fees from Novo Nordisk, Kiniksa, and Horizon Therapeutics (now Amgen) for unrelated work, and is supported by NIH/NHLBI K23HL159276 and American Heart Association 21CDA851511. Dr Osborne receives consulting fees from WCG Clinical for unrelated work, and is supported by the NIH K23HL151909 and the American Heart Association 23SCISA1143491. Dr Bucerius receives institutional funding from the IAEA in the context of the PIAF (Prognostic Value of Arterial 18F-FDG PET Imaging in Patients with History of Myocardial Infarction) trial (core laboratory). Dr Unizony reports Research support from Genentech, NIH 1UM1AI144295-01 for unrelated work, and consulting fees from Novartis, Sanofi, and Harvard Pilgrim Health Care. The other authors report no conflicts.

Vascular imaging is an integral part of large-vessel vasculitis (LVV) evaluation and management. Several imaging modalities are currently employed in clinical practice including vascular ultrasound, computed tomography angiography, MRI and magnetic resonance angiography, and 18 F-fluorodeoxyglucose PET. Well-established roles for imaging in LVV include disease diagnosis and assessment of luminal lesions reflecting vascular damage. The ability of imaging to determine treatment response, monitor disease activity, and predict future arterial damage is an area of active research., Competing Interests: Disclosure M.A. Matza. None. S. Unizony: Consulting: Harvard Pilgrim Health Care, IQVIA, and Sanofi. Research support: Genentech. A.B. Arevalo: Consulting: AMGEN., (Copyright © 2024 Elsevier Inc. All rights reserved.)

This study examines the characteristics of patients with giant cell arteritis (GCA), the utilization of imaging in GCA diagnosis, and variations in GCA management among specialties. Subjects were identified from the Dallas VAMC database spanning 2010 to 2021 using ICD-9/10 codes for GCA and polymyalgia rheumatica, and a list of temporal artery biopsies (TAB). Patients lacking sufficient data to meet the ACR 1990 classification criteria for GCA were excluded. Categorical variables were compared using Fisher's exact test. Continuous variables were analyzed with the Kruskal-Wallis test. Among 209 identified patients, 41 were excluded due to insufficient data for ACR classification. The cohort comprised 91.9% males with a median age of 69. Of the remaining 168 patients, 42 received a final diagnosis of GCA, and 15 of these were confirmed with a positive TAB. The most reported initial symptoms were visual disturbances (75.5%) and headaches (67.7%). Ophthalmology was the initial physician for 46% of patients. GCA correlated with co-existing autoimmune diseases, glucocorticoid-sparing treatments, and consultation with a rheumatologist (p < 0.05). There were no significant differences in clinical features or management of the positive and negative TAB GCA groups. GCA presents with heterogeneous symptoms making diagnosis challenging. Scalp tenderness and headaches were significantly higher in GCA patients, but sub-group analysis revealed no significant differences among GCA patients. Vascular assessments and adjunct imaging modalities are underutilized. The establishment of multidisciplinary or fast-track clinics may enhance the optimization of GCA management. Key Points • The most common presenting symptoms were blurry vision/visual loss (75.5%), headache (67.7%), and scalp tenderness (35.9%) in descending order. • In sub-group analysis, no significant differences were found between GCA sub-groups, but when compared to the non-GCA group, were found to have significantly higher rates of headache and scalp tenderness. • Compared to other specialties, rheumatologists were more likely to use advanced imaging, and to prescribe glucocorticoid-sparing treatments. • Systematic and comprehensive assessment and multidisciplinary approach could improve diagnosis and management., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Background/objectives: Giant cell arteritis (GCA) is an inflammatory vascular disease in which prompt and accurate diagnosis is critical. The efficacy of temporal artery biopsy (TAB) is limited by 'skip' lesions and a delay in histological analysis. This first-in-man ex-vivo study aims to assess the accuracy of optical frequency domain imaging (OFDI) in diagnosing GCA., Subjects/methods: 29 TAB samples of patients with suspected GCA were submerged in 0.9% sodium chloride and an OFDI catheter was passed through the lumen to create cross-sectional images prior to histological analysis. The specimens were then preserved in formalin for histological examination. Mean intimal thickness (MIT) on OFDI was measured, and the presence of both multinucleate giant cells (MNGCs) and fragmentation of the internal elastic lamina (FIEL) was assessed and compared with histology, used as the diagnostic gold standard., Results: MIT in patients with/without histological evidence of GCA was 0.425 mm (±0.43) and 0.13 mm (±0.06) respectively compared with 0.215 mm (±0.09) and 0.135 mm (±0.07) on OFDI. MIT measured by OFDI was significantly higher in patients with histologically diagnosed arteritis compared to those without (p = 0.0195). For detecting FIEL and MNGCs, OFDI had a sensitivity of 75% and 28.6% and a specificity of 100% and 77.3% respectively. Applying diagnostic criteria of MIT > 0.20 mm, or the presence of MNGCs or FIEL, the sensitivity of detecting histological arteritis using OFDI was 91.4% and the specificity 94.1%., Conclusions: OFDI provided rapid imaging of TAB specimens achieving a diagnostic accuracy comparable to histological examination. In-vivo imaging may allow imaging of a longer arterial section., (© 2024. The Author(s).)

Objectives: The aim is to access the real-world clinical management of physicians who treat Takayasu arteritis (TAK) and giant cell arteritis (GCA) after the publication of the Japanese Circulation Society (JCS) 2017 Guidelines for the Management of Vasculitis Syndrome., Methods: This descriptive, cross-sectional study utilized self-administered electronic questionnaires, which were answered in February 2022 by physicians treating TAK or GCA and registered with Macromill Inc., Results: The 329 survey respondents were enrolled. The 2017 JCS Guidelines were the most commonly referenced information source for resolving clinical questions, accessed by 70% of respondents. Ophthalmoscopy was performed in only 50% of patients with TAK and in 70% for GCA. The median percentages of patients who underwent 18F-fluorodeoxyglucose-positron emission tomography/computed tomography for TAK and GCA patients were 23% and 20% at diagnosis, respectively, and 10% each at follow-up within 12 months. Tocilizumab was the most frequently used medication in combination with glucocorticoids for both TAK and GCA, especially in remission induction therapy for relapsed patients., Conclusions: The majority of physicians treating TAK and GCA referred to the 2017 JCS guidelines. This report clarified the current clinical practice for large vessel vasculitis in Japan, providing information for the next revision of the guidelines., (© Japan College of Rheumatology 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com.)

In the recent EULAR recommendations, ultrasound examination is now recommended as a first-line imaging test in all patients with suspected giant cell arteritis (GCA) and the axillary arteries should be included in the standard exam. As an alternative to ultrasound evaluation, cranial and extracranial arteries can be examined using FDG-PET or MRI. The aim of our study was to observe in a retrospective case series whether there is a correlation between biomarkers and imaging activity in a population of patients followed in real life with GCA treated with prednisone (PDN) and tocilizumab (TCZ). We retrospectively enrolled 68 patients with newly diagnosed GCA between January 2020 and September 2021, followed in real life, who were examined at the Rheumatology Unit of the San Giovanni di Dio Hospital, Florence, Italy. Patients were evaluated at T0-T3-T6-T12-T18-T24 for the following blood tests: ESR, CRP, fibrinogen, platelet count, serum amyloid A (SAA), IL-6, and circulating calprotectin (MRP). Ultrasound examination of the temporal arteries and axillary arteries was assessed at T0 within 7 days of starting treatment with high-dose glucocorticoids and subsequently at T3-T6-T12-T18-T24. A scale from 0 to 3 with semi-quantitative tools (SUV max) was assessed at T0-T12-T24. The evaluation of the correlation coefficient between laboratory and imaging variables has shown that SAA and MRP have the most powerful correlation with the PET score (0.523 and 0.64), and MRP also has an excellent correlation coefficient with the Halo score (0.658). The evaluation of the ROC curves shows for a PET score 3 and SAA values higher than 26 mg/L, sensitivity of 81.5% and specificity of 84.1%, and for a PET score 3 and MRP values higher than 2.3 mcg/mL, sensitivity of 100% and specificity of 76.8%. In this study, we demonstrated that SAA and MRP can be useful as promising tools to detect GCA activity. The study demonstrates a good correlation between the two biomarkers and the imaging activity evaluated by the Halo and PET scores., Competing Interests: Declarations Conflict of interest The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Objectives: Our aim was to introduce a standardized system for assessing the extent of GCA on MRI, i.e. the Magnetic Resonance Vasculitis Activity Score (MRVAS). To obtain a comprehensive view, we used an extensive MRI protocol including cranial vessels and the aorta with its branches. To test reliability, MRI was assessed by four readers with different levels of experience., Methods: A total of 80 patients with suspected GCA underwent MRI of the cranial arteries and the aorta and its branches (20 vessel segments). Every vessel was rated dichotomous [inflamed (coded as 1) or not (coded as 0)], providing a summed score of 0-20. Blinded readers [two experienced radiologists (ExR) and two inexperienced radiologists (InR)] applied the MRVAS on an individual vessel and an overall level (defined as the highest score of any of the individual vessel scores). To determine interrater agreement, Cohen's κ was calculated for pairwise comparison of each reader for individual vessel segments. Intraclass correlation coefficients (ICCs) were used for the MRVAS., Results: Concordance rates were excellent for both subcohorts on an individual vessel-based (GCA: ICC 0.95; non-GCA: ICC 0.96) and overall MRVAS level (GCA: ICC 0.96; non-GCA: ICC 1.0). Interrater agreement yielded significant concordance (P < 0.001) for all pairs (κ range 0.78-0.98). No significant differences between ExRs and InRs were observed (P = 0.38)., Conclusion: The proposed MRVAS allows standardized scoring of inflammation in GCA and achieved high agreement rates in a prospective setting., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction/objectives: Optic nerve sheath (ONS) enhancement using magnetic resonance imaging of the orbits was observed in patients with giant cell arteritis (GCA). We previously showed that ONS diameter (ONSD) by bedside ultrasound is increased in patient with active GCA. This study aims to assess whether ONSD decreases with clinical remission in patients with GCA., Methods: A prospective cohort study was conducted from June 2022 to January 2023. Patients who had an optic nerve ultrasound at GCA diagnosis as part of a previous crosssectional study were eligible. Optic nerve ultrasound was performed by the same investigator at diagnosis and month 3. ONSD (includes the optic nerve and its sheath) and optic nerve diameter (OND) were measured. Descriptive statistics for baseline characteristics and paired sample t-test were performed to assess the mean difference in OND and ONSD between diagnosis and month 3., Results: Nine patients with GCA were included. The median age at disease onset was 79 years (interquartile range (IQR) of 79-82 years), and 7 patients were males. All patients were in clinical remission at month 3 on prednisone (median dose of 15 mg/day, IQR of 10-25 mg). The mean ONSD was lower at month 3 (3.76 mm) compared to baseline (5.98 mm), with a paired mean difference of 2.22 mm (95% CI 1.41-3.03 mm, p < 0.001). As anticipated, OND measurements did not vary between diagnosis and month 3., Conclusion: ONSD on ultrasound improves after 3 months of therapy in patients with GCA. A longer prospective study is required to determine if ONSD is useful to assess disease activity in GCA. Key Points • ONS ultrasound can identify patients with active GCA. • The ONSD on ultrasound is dynamic and improved after 3 months of GCA therapy. • ONS ultrasound may be useful to monitor disease activity in GCA., (© 2024. The Author(s).)

Objective: Conventional two-dimensional ultrasound has been assessed for the non-invasive diagnosis of giant cell arteritis (GCA), but the results are operator dependent, resulting in low sensitivity. Tomographic three-dimensional (3D) ultrasound is a novel technique that enables the objective documentation of vessel geometry. Here, for the first time, its utility is assessed for visualizing temporal arteries., Method: The temporal artery of 14 healthy subjects and three subjects with suspected GCA was examined using tomographic 3D ultrasound., Results: This technique enabled 3D mapping of the architecture of the temporal artery. The inner and outer vessel diameters showed considerable interindividual variability. However, calculation of the vessel wall fraction revealed the combination of vessel wall thickening and lumen narrowing, which may be indicative of GCA., Conclusions: This proof-of-concept study indicates that tomographic 3D ultrasound can be used for objective mapping of the temporal artery. The technique must be evaluated regarding its diagnostic sensitivity in GCA before it can be introduced in clinical practice.

Objectives: Giant cell arteritis (GCA) is the main systemic vasculitis in individuals aged ≥ 50 years. Color Doppler ultrasound (CDS) has an established role in GCA diagnosis and management. This study aims to assess the clinical characteristics associated with a positive CDS evaluation and the impact of additional axillary artery examination on diagnostic sensitivity., Material and Methods: We conducted a retrospective analysis of patients undergoing CDS of the superficial temporal arteries, with or without axillary artery assessment, at our hospital, between 2009 and 2023. Patients meeting the new 2022 diagnostic criteria for GCA were included and their characteristics were analyzed according to the presence of the halo sign on CDS., Results: Of the 135 included patients (54 % female, mean age 75 ± 8 years), the halo sign was observed in 57 %, correlating with higher systemic symptom prevalence (61 % vs 42 %, p = 0.035), lower hemoglobin (p < 0.001), and higher erythrocyte sedimentation rate (p = 0.028). The halo sign inversely related to prior corticosteroid therapy (p = 0.033). Patients with axillary halo sign had fewer external carotid symptoms and a higher vertebral halo sign prevalence. Vertebral halo sign was associated with posterior circulation ischemic stroke (65 %, p < 0.001). Axillary artery studies improved diagnostic sensitivity by 9 %., Conclusion: In our study, the halo sign correlated with higher systemic symptoms and analytical abnormalities. Axillary artery examination enhanced CDS sensitivity, linked to severe outcomes like stroke. Prior corticosteroid therapy reduced CDS sensitivity. The correlation of clinical, laboratory, and ultrasound findings provides a more comprehensive understanding of GCA pathogenesis and evolution., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: The objective of this study was to prospectively evaluate the diagnostic efficacy of transorbital ultrasound (TOS) in patients newly diagnosed with giant cell arteritis (GCA), presenting with visual symptoms., Methods: Patients with newly diagnosed, untreated GCA were examined using TOS, assessing central retinal artery flow velocity [peak systolic velocity (PSV), end-diastolic velocity (EDV), resistance index (RI)], and optic nerve diameter (OND). Vascular ultrasound was conducted to evaluate the superficial temporal arteries, their branches, facial, axillary, carotid, and vertebral arteries., Results: We enrolled 54 GCA patients, 27 with visual symptoms, and 27 healthy controls. Eyes of GCA patients with visual symptoms demonstrated significantly lower PSV and EDV (PSV: β = -1.91; P = 0.029; EDV: β = -0.57; P = 0.032) and significantly elevated OND (β = 0.79; P = 0.003) compared with controls. RI did not significantly differ from controls (β = -0.06, P = 0.129). Vascular ultrasound identified an average of 8.7 (SD ± 2.8) pathological vessels per GCA patient. A significant negative association was observed between the number of affected vessels and both PSV (P = 0.048) and EDV (P = 0.040). No association was found with RI (P = 0.249), while a positive significant association was noted with OND (P < 0.001)., Conclusions: This study pioneers the application of TOS to assess structural eye changes in newly diagnosed, untreated GCA patients with visual symptoms. Our findings suggest reduced central retinal artery flow and increased optic nerve diameter as potential biomarkers for serious ocular involvement in GCA. The detected association between internal and external carotid artery involvement indicates a common pathophysiological mechanism underlying systemic and ocular manifestations of GCA., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: Vascular ultrasound is commonly used to diagnose giant cell arteritis (GCA). Most protocols include the temporal arteries and axillary arteries, but it is unclear which other arteries should be included. This study investigated whether inclusion of intima media thickness (IMT) of the common carotid artery (CCA) in the ultrasound evaluation of GCA improves the accuracy of the examination., Methods: We formed a fast-track clinic to use ultrasound to rapidly evaluate patients with suspected GCA. In this cohort study, patients referred for new concern for GCA received a vascular ultrasound for GCA with the temporal arteries and branches, the axillary artery, and CCA., Results: We compared 57 patients with GCA and 86 patients without GCA. Three patients with GCA had isolated positive CCA between 1 and 1.49 mm, and 21 patients without GCA had isolated positive CCA IMT. At the 1.5-mm CCA cutoff, 4 patients without GCA had positive isolated CCA, and 1 patient with GCA had a positive isolated CCA. The sensitivity of ultrasound when adding carotid arteries to temporal and axillary arteries was 84.21% and specificity 65.12% at an intima media thickness (IMT) cutoff of ≥1 mm and 80.70% and 87.21%, respectively, at a cutoff of ≥1.5 mm., Conclusion: Measurement of the CCA IMT rarely contributed to the diagnosis of GCA and increased the rate of false-positive results. Our data suggest that the CCA should be excluded in the initial vascular artery ultrasound protocol for diagnosing GCA. If included, an IMT cutoff of higher than 1.0 mm should be used., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Steroids and anti-IL6 biotherapy are highly effective in obtaining remission in patients with giant cell arteritis (GCA) but the risk of relapses remains high. We aimed to identify predictors of relapse in GCA., Methods: All consecutive patients admitted with a new diagnosis of GCA - according to the 2022 American College of Rheumatology/EULAR (ACR/EULAR) classification criteria - between May 2011 and May 2022 were eligible for this study. The primary outcome was the GCA relapse rate over the 36-months follow up. Factors associated with the primary outcome and time to first relapse were analyzed., Results: One hundred and eight patients (74 [69-81] years, 64.8% women) with a new diagnosis of GCA were studied. GCA was biopsy-proven in 65 (60.2%) cases. Ninety-eight (90.7%) FDG/PET CT scans performed at diagnosis were available for review. All patients received steroids given for 21.0 [18.0-28.5] months, associated with methotrexate (n=1, 0.9%) or tocilizumab (n=2, 1.9%). During a median follow-up of 27.5 [11.4-35.0] months, relapse occurred in 40 (37%) patients. Multivariable Cox regression model, including general signs, gender, aortic wall thickness, FDG uptake in arterial wall and IV steroid pulse as covariates, showed that both general signs (HR 2.0 [1.0-4.0, P<0.05) and FDG uptake in limb arteries (HR 2.7 [1.3-5.5], P<0.01) at diagnosis were associated with GCA relapse., Conclusion: FDG uptake in limb arteries at diagnosis is a predictor of relapse in newly diagnosed GCA., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Objective: Giant cell arteritis (GCA) is the most common vasculitis and can result in blindness due to anterior ischemic optic neuropathy (AION). Little is known about which patients with GCA are at higher risk of AION.  We did a retrospective chart review to compare demographics, past medical history, labs and imaging of GCA patients with and without AION., Methods: All patients at the University of Washington who were diagnosed with GCA by a rheumatologist, had a vascular ultrasound and met classification criteria for GCA were included. AION was diagnosed by an ophthalmologist. To compare demographics, symptoms, presentation imaging and lab findings, we used Pearson Chi-square, Fisher's exact test and t-tests to compare GCA patients with and without AION., Results: 91 patients with GCA without vision loss were compared to 15 patients with GCA and AION. The AION group had significantly more men (p=0.03) and elevated C-reactive protein (CRP) as compared to the non-AION group (p = 0.04). Rates of hypertension, smoking, erythrocyte sedimentation rate (ESR) and classification scores were similar. Vascular ultrasound showed similar rates of small and large vessel vasculitis, but the AION group had higher halo scores, with the AION group having a mean score of 5 and patients with GCA and without AION having a mean score of 3 (p < 0.01)., Conclusions: Older men with GCA and more than three halos on vascular ultrasound imaging of GC may be at higher risk of AION. Key Points • This is the first paper to incorporate the use of vascular ultrasound in comparing patients with anterior ischemic optic neuropathy (AION) to those without giant cell arteritis (GCA). • Male sex is associated with AION despite GCA being more common in women. • Older age and higher CRP are associated with AION. • GCA patients with AION had a higher halo score than GCA patients without AION., Competing Interests: Compliance with ethical standards. Disclosures: None., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Introduction: To reach the diagnosis of giant cell arteritis (GCA), signs, symptoms, laboratory tests, imaging findings, and occasionally anatomopathological results from temporal artery biopsy are evaluated. This study describes the results of an algorithm analysis based on clinical and ultrasound evaluation of patients with suspected GCA, highlighting its diagnostic utility by contrasting its use in different clinical suspicion scenarios., Method: Prospective multicenter study evaluating patients referred with suspected GCA through a preferential circuit (fast track), grouping them according to low or high clinical suspicion of GCA. Each of these scenarios is evaluated by biopsy and ultrasound for all patients, resulting in positive, indeterminate, or negative outcomes, yielding six possible groups. Potential areas of improvement are explored, emphasizing that, following a negative or indeterminate ultrasound, 18-FDG-PET-CT could be recommended. We analyze the results and application of a diagnostic algorithm, confirming its efficiency and applicability based on whether there is high or low clinical suspicion., Results: Sixty-nine patients (41 in the high suspicion group and 28 in the low suspicion group). There were 41 new diagnoses of GCA: 35 in the high suspicion group and 6 in the low suspicion group. Using ultrasound alone, the initial algorithm has an overall diagnostic efficiency of 72.5%, which improves to 80.5% when including 18F-FDG-PET/CT. The negative predictive value of ultrasound in patients with low clinical suspicion is 84.6%, and the positive predictive value of ultrasound in patients with high suspicion is 100%, improving sensitivity from 57.1% to 80.8% with 18F-FDG-PET/CT in this scenario. Temporal artery biopsy was performed on all patients, with no differences in sensitivity or specificity compared to ultrasound. In cases where all three tests - ultrasound, biopsy, and 18F-FDG-PET/CT - are performed, sensitivity increases to 92.3% in patients with high clinical suspicion., Conclusion: In situations of high clinical suspicion, the algorithm provides sufficient information for the diagnosis of GCA if ultrasound is positive. A negative ultrasound is sufficient to rule out the diagnosis in the context of low clinical suspicion. 18-FDG-PET-CT may be useful in patients with high suspicion and negative or indeterminate ultrasound results., (Copyright © 2024 The Authors. Published by Elsevier España, S.L.U. All rights reserved.)

Objective: Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using 18 F-FDG PET/CT in patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA)., Materials and Methods: PubMed and EMBASE were searched and the results were screened by two reviewers. Study quality was assessed using a modified version of the Newcastle-Ottawa scale. Heterogeneity between studies was assessed using the I 2 statistic and the Q test. Further subgroup analyses were performed by disease type, study quality, and 18 F-FDG PET/CT uptake criteria. Publication bias was assessed by funnel plot and Egger's test., Results: 268 publications were identified, of which 17 met the selection criteria and were included in the meta-analysis. The overall pooled prevalence of extracranial LVV by 18 F-FDG PET/CT was 54.5% [95% CI: 42.6%-66.1%]. In patients with GCA the prevalence was significantly higher than in patients with PMR (60.1% vs. 41.8%, P = 0.006). Likewise, studies with a lower risk of bias reported a higher prevalence of extracranial LVV (61.1% vs. 46.9%; P = 0.010). No publication bias was observed., Conclusions: The 18 F-FDG PET/CT test may be useful in the detection of extracranial LVV, both in patients with PMR or GCA. Such involvement is more frequent in patients with GCA, and may vary depending on the quality of the studies., (Copyright © 2024 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.)

Objectives: To evaluate the ability of 18 FDG PET/CT, at diagnosis of giant cell arteritis (GCA) and during follow-up, to predict occurrence of relapse in large-vessel GCA (LV-GCA)., Methods: We conducted a retrospective study using the French Study Group for Large-Vessel Vasculitis (GEFA) network. Data from patients with LV-GCA diagnosed by PET/CT and who had PET/CT in the following year were collected. For each PET/CT, PET vascular activity score (PETVAS) and total vascular score (TVS) were assessed, and their ability to predict the occurrence of subsequent relapse was assessed., Results: A total of 65 LV-GCA patients were included, of whom 55 had undergone a follow-up PET/CT 3 to 12 months after the diagnosis of GCA. Patients for whom the second PET/CT (PET2) was performed during active GCA were excluded. PETVAS and TVS decreased between PET1 and PET2 in all patients (p < 0.001). There was no correlation between vascular activity scores in PET2 and time to prednisone taper. For relapse prediction, at PET1, the AUC of the TVS and PETVAS were respectively 51.9 and 41.9 at 6 months, 55.3 and 49.7 at 1 year, 55 and 55.7 at 2 years. For PET2, the AUC were respectively 46.1 and 46.7 at 6 months, 52.1 and 48.9 at 1 year, 58.4 and 52.3 at 2 years., Conclusion: PET vascular activity scores at diagnosis and at follow-up PET/CT performed outside a period of GCA activity do not display high performance to predict the occurrence of subsequent relapse in LV-GCA patients., Competing Interests: Disclosures Maxime Samson: Argenx (consulting), Boerhinger Ingelheim (consulting), Chugai (consulting), CSL Vifor (consulting), Fresenius Kabi (consulting), GSK (consulting), Novartis (consulting and research grant), (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: To evaluate the reproducibility of vessel wall magnetic resonance imaging (VW-MRI) in diagnosing giant cell arteritis (GCA) among groups of radiologists with varying levels of expertise., Methods: This institutional review board-approved retrospective single-center study recruited patients with suspected GCA between December 2014 and September 2021. Patients underwent 3 -T VW-MRI before temporal artery biopsy. Ten radiologists with varying levels of expertise, blinded to all data, evaluated several intracranial and extracranial arteries to assess GCA diagnosis. Interobserver reproducibility and diagnostic performance were evaluated., Results: Fifty patients (27 women and 23 men) with a mean age of 75.9 ± 9 years were included. Thirty-one of 50 (62%) had a final diagnosis of GCA.VW-MRI had an almost perfect reproducibility among expert readers (kappa = 0.93; 95% CI 0.77-1) and substantial reproducibility among all readers, junior and non-expert senior readers (kappa = 0.7; 95% CI 0.66-0.73; kappa = 0.67 95% CI 0.59-0.74; kappa = 0.65; 95% CI 0.43-0.88 respectively) when diagnosing GCA. Substantial interobserver agreement was observed for the frontal branch of superficial temporal artery. Moderate interobserver agreement was observed for the superficial temporal artery and its parietal branch, as well as ophthalmic arteries in all groups of readers. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy varied depending on the group of readers., Conclusion: VW-MRI is a reproducible and accurate imaging modality for detecting GCA, even among less-experienced readers. This study advocates for the use of VW-MRI when diagnosing GCA even in less-experienced centers., Clinical Relevance Statement: VW-MRI is a reproducible and accurate imaging modality for detecting GCA, even among less-experienced readers, and it could be used as a first-line diagnostic tool for GCA in centers with limited expertise in GCA diagnosis., Key Points: • Vessel wall magnetic resonance imaging (VW-MRI) is a reproducible and accurate imaging modality for detecting giant cell arteritis (GCA) in both extracranial and intracranial arteries. • The reproducibility of vessel wall magnetic resonance imaging for giant cell arteritis diagnosis was high among expert readers and moderate among less-experienced readers. • The use of vessel wall magnetic resonance imaging for giant cell arteritis diagnosis can be recommended even in centers with less-experienced readers., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

The aim of the study was to evaluate the clinical manifestations and survival of patients with giant cell arteritis (GCA)., Materials and Methods: . A retrospective study included 166 patients with newly diagnosed GCA. Clinical, laboratory, and instrumental data and three sets of classification criteria were used to confirm the diagnosis: the American College of Rheumatology (ACR) 1990, the revised ACR criteria of 2016 and/or the new ACR and European Alliance of Rheumatologic Associations (EULAR) 2022 criteria. Some of the patients underwent instrumental investigations: temporal artery ultrasound Doppler (n = 61), contrast-enhanced computed tomography (n = 5), CT angiography (n = 6), magnetic resonance imaging (n = 4), MR angiography (n = 3), and 18F-FDG PET/CT (n = 47). Overall and recurrence-free survival rates were analyzed using survival tables and Kaplan-Meier method., Results: . The most frequent first manifestations of GCA were headache (81.8%), weakness (64%), fever (63.8%), and symptoms of rheumatic polymyalgia (56.6%). Changes in temporal arteries in color duplex scanning were detected in 44 out of 61 patients. GCs therapy was performed in all patients who agreed to be treated (n = 158), methotrexate was used in 49 out of 158 patients, leflunomide in 9 patients. In 45 (28.5%) out of 158 patients, a stable remission was achieved as a result of GC monotherapy; in 120 (75.9%) patients, long-term maintenance therapy with GCs was required to prevent exacerbations, including 71 (44.9%) patients in combination with methotrexate or other immunosuppressive drugs. The follow-up period of patients with a history of relapses was 21.0 (8.0-54.0) months. Relapses developed in 73 (46.2%) patients. The overall one-year survival rate was 97.1% [95% CI 94.3; 99.9], and the five-year survival rate of patients was 94.6% [95% CI 90.2; 99.0]. The one-year relapse-free survival rate was 86.4% [95% CI 80.5; 92.3], and the five-year relapse-free survival rate was 52.4% [95% CI 42.0; 62.8]. Twelve (7.2%) of 166 patients died. The cause of death was myocardial infarction in two patients, stroke in two patients, and breast cancer in one patient; in the remaining seven cases, the cause of death was not determined., Conclusions: : Given the high frequency of disease exacerbation, patients with GCA require long-term follow-up, especially during the first year after diagnosis., (© 2024. Pleiades Publishing, Ltd.)

There is a growing appreciation that both giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely interrelated conditions that have significant overlap in aetiology, clinical characteristics and treatment regimens. Subclinical GCA in PMR is becoming increasingly recognised, and there is evolving evidence that this may be a more aggressive disease phenotype than PMR. Ultrasound (US) lends itself well as a screening tool for GCA in PMR; it is inexpensive, non-invasive, widely available, lacks ionising radiation, may be performed at the bedside and is recommended by EULAR as a first-line investigation for suspected GCA. There is insufficient evidence to currently recommend that all patients with PMR should have a US assessment for vascular involvement. However, as clinical and laboratory parameters alone do not accurately diagnose patients with subclinical GCA, we suggest that vascular US will be increasingly performed by rheumatologists in practice to identify these patients with PMR, preferably as part of larger prospective outcome studies., Competing Interests: Competing interests: SC: grant support from Novatris, speaker honoria from Janssen and conference attendance support from Abbvie, Janssen and Novartis. CK: conference attendance support from Novartis. PH: grant support from Janssen and Novartis and conference attendance support from Abbvie and Novartis. RC: grant support from Janssen and Novartis and consulting fees/speaker fees from Janssen, Abbvie, Fresenius Kabi, Galapagos, UCB, Viatris, Celltrion, Nordic Pharma and Novartis. DJK: grant support from Novartis and conference attendance support from Abbvie., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ on behalf of EULAR.)

Different types of vasculitis can be distinguished according to the blood vessel's size that is preferentially affected: large-vessel, medium-vessel, and small-vessel vasculitides. Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are the main forms of large-vessel vasculitis, and may lead to lumen narrowing. Clinical manifestations of arterial narrowing on the short- and long term include vision loss, stroke, limb ischemia, and heart failure. Imaging tools are well established diagnostic tests for large-vessel vasculitis and may aid therapy monitoring in selected cases while providing important information regarding the occurrence of vascular damage, tissue and organ complications. This review aims to provide the current status of multimodality imaging for the diagnosis and identification of vascular complications in the field of large vessel vasculitis., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: While early diagnosis of giant cell arteritis (GCA) based on clinical criteria and contrast-enhanced MRI findings can lead to early treatment and prevention of blindness and cerebrovascular accidents, previously reported diagnostic methods which utilize contrast-enhanced whole head images are cumbersome. Diagnostic delay is common as patients may not be aware of initial symptoms and their significance. To improve current diagnostic capabilities, new MRI-based diagnostic criteria need to be established. This study aimed to evaluate the "multifocal arcuate sign" on short tau inversion recovery (STIR) and contrast-enhanced T1-weighted (CE-T1W) images as a novel extracranial finding for the diagnosis of GCA., Methods: A total of 17 consecutive patients (including five with GCA) who underwent CE-T1W and whole-brain axial STIR imaging simultaneously between June 2010 and April 2020 were enrolled. We retrospectively reviewed their MR images. The "multifocal arcuate sign" was defined as "multiple distant arcuate areas with high signal intensity in extracranial soft tissues such as subcutaneous fat, muscles, and tendons." Extracranial abnormal high-signal-intensity areas were classified as "None," when no lesions were detected; "Monofocal," when lesions were detected only in one place; and "Multifocal," when lesions were detected in multiple places. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of "Multifocal" areas were calculated using cross tabulation. Fisher's exact test was used to compare "Multifocal" areas in five patients with GCA and those with other diseases. In addition, mean Cohen's kappa and Fleiss' kappa statistics were used to compare inter-reader agreement., Results: The sensitivity, specificity, PPV, and NPV of the "multifocal arcuate sign" in patients with GCA were 60%, 92-100%, 75-100%, and 85-86%, respectively. Significantly more patients with GCA had "Multifocal" areas compared to those with other diseases (Fisher's exact test, p = 0.008-0.027). Mean Cohen's kappa and Fleiss' kappa for inter-reader agreement with respect to the five GCA patients were 0.52 and 0.49, respectively, for both STIR and CE-T1W sequences., Conclusions: The new radiologic finding of "multifocal arcuate sign" on STIR and CE-T1W images may be used as a radiologic criterion for the diagnosis of GCA, which can make plain MRI a promising diagnostic modality., (© 2024. The Author(s).)

Large vessel vasculitis (LVV) affects mainly large arteries with giant cell arteritis (GCA) and Takayasu arteritis (TAK) being the two most frequent forms. Clinical symptoms can be non-specific, including headache, fatigue, weight loss, and change in vision. Untreated, LVV may also lead to serious complications such as blindness, aortic aneurysm and dissection. Therefore, rapid recognition of the disease leading to accurate diagnosis and appropriate treatment is essential. FDG-PET/CT imaging has emerged as a sensitive marker of active vascular inflammation and its use in the management of LVV is now integrated in guidelines. In this article, we will discuss the role of FDG-PET/CT for the diagnosis of LVV and monitoring of therapy, as well as review technical and interpretation parameters., (Copyright © 2023. Published by Elsevier Inc.)

Objective: Fluorine-18-fluorodeoxyglucose (18F-FDG) PET/CT is a promising diagnostic tool for polymyalgia rheumatica (PMR) and large vessel vasculitis (LVV). PET/CT performance is recommended before the onset of steroid therapy because glucocorticoids (GC) may decrease the intensity of FDG uptake. However, this is not always possible in clinical practice. Our aim was to assess if PET/CT could be also useful to detect musculoskeletal and vascular involvement in patients receiving GC., Methods: Single-center study of patients with PMR diagnosis based on 2012 EULAR/ACR criteria who underwent a PET/CT scan due to LVV suspicion. We compared the musculoskeletal and vascular FDG uptake between two groups: (a) steroid-naïve and (b) steroid-resistant patients. A sub-analysis was conducted in patients who were receiving GC to discern if the cumulative prednisone dose influences the FDG uptake., Results: We evaluated 75 patients (27 men/ 48 women); mean age±SD: 68.2 ± 10.7 years. PET/CT was performed in 14 steroid-naïve and 61 steroid-resistant patients. Patients under GC had received a median cumulative prednisone dose of 1.8 [0.6-3.9] g. The pattern of musculoskeletal FDG uptake was similar in steroid-naïve and steroid-resistant patients. FDG uptake in the vessel wall was more frequently detected in steroid-naïve patients. However, PET/ CT was also useful to detect LVV in 62.3 % of the patients who were receiving GC. The percentage of patients who had positive PET/CT scans tended to decrease with higher cumulative prednisone doses., Conclusion: Even though GC therapy may decrease the 18-FDG uptake, PET/CT continues to be a useful tool to detect musculoskeletal and LVV involvement in PMR., (Copyright © 2024 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

Background: Giant cell arteritis (GCA) is the most prevalent systemic vasculitis in people older than 50 years. Any delay in diagnosis impairs patients' quality of life and can lead to permanent damage, particularly vision loss., Objective: To evaluate a diagnostic strategy for GCA using color Doppler ultrasound of the temporal artery as a first-line diagnostic test, temporal artery biopsy (TAB) as a secondary test, and physician expertise as the reference method., Design: Prospective multicenter study with a 2-year follow-up. (ClinicalTrials.gov: NCT02703922)., Setting: Patients were referred by their general practitioner or ophthalmologist to a physician with extensive experience in GCA diagnosis and management in one of the participating centers: 4 general and 2 university hospitals., Patients: 165 patients with high clinical suspicion of GCA, aged 79 years (IQR, 73 to 85 years)., Intervention: The diagnostic procedure was ultrasound, performed less than 7 days after initiation of corticosteroid therapy. Only ultrasound-negative patients underwent TAB., Measurements: Bilateral temporal halo signs seen on ultrasound were considered positive. Ultrasound and TAB results were compared with physician-diagnosed GCA based on clinical findings and other imaging., Results: Diagnosis of GCA was confirmed in 44%, 17%, and 21% of patients by ultrasound, TAB, and clinical expertise and/or other imaging tests, respectively. Their diagnosis remained unchanged at 1 month, and 2 years for those with available follow-up data. An alternative diagnosis was made in 18% of patients. The proportion of ultrasound-positive patients among patients with a clinical GCA diagnosis was 54% (95% CI, 45% to 62%)., Limitation: Small sample size, no blinding of ultrasound and TAB results, lack of an objective gold-standard comparator, and single diagnostic strategy., Conclusion: By using ultrasound of the temporal arteries as a first-line diagnostic tool in patients with high clinical suspicion of GCA, further diagnostic tests for patients with positive ultrasound were avoided., Primary Funding Source: Tender "Recherche CH-CHU Poitou-Charentes 2014.", Competing Interests: Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M23-3417.

Competing Interests: The authors report no conflicts of interest.

Giant cell arteritis is the principal form of systemic vasculitis affecting people over 50. Large-vessel involvement, termed large vessel giant cell arteritis, mainly affects the aorta and its branches, often occurring alongside cranial giant cell arteritis, but large vessel giant cell arteritis without cranial giant cell arteritis can also occur. Patients mostly present with constitutional symptoms, with localising large vessel giant cell arteritis symptoms present in a minority of patients only. Large vessel giant cell arteritis is usually overlooked until clinicians seek to exclude it with imaging by ultrasonography, magnetic resonance angiography (MRA), computed tomography angiography (CTA), or [ 18 F]fluorodeoxyglucose-PET-CT. Although the role of imaging in treatment monitoring remains uncertain, imaging by MRA or CTA is crucial for identifying aortic aneurysm formation during patient follow up. In this Series paper, we define the large vessel subset of giant cell arteritis and summarise its clinical challenges. Furthermore, we identify areas for future research regarding the management of large vessel giant cell arteritis., Competing Interests: Declaration of interests KSMvdG reports a grant from FOREUM Foundation for Research in Rheumatology, speaker fees from Roche, and research support from AbbVie, all outside the submitted work and paid to the University Medical Center Groningen (UMCG). TAB reports research grants from Deutsche Forschungsgemeinschaft and Siemens Healthineers paid to his institution and personal consulting fees and honoraria for lectures from Bracco, BTR, Guerbet, Siemens Healthineers, Novartis, and Roche. RHJAS reports independent research grants from Siemens Healthineers and Pfizer. EB, as an employee of the UMCG, received grants from the Dutch Arthritis Society and the EU, European Federation of Pharmaceutical Industries and Associations, and Innovative Medicines Initiative 2 Joint Undertaking Immune-Image (grant no 831514), which were paid to the UMCG. All other authors declare no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Objectives: To investigate the value of [ 18 F]fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) in predicting relapse after treatment discontinuation in patients with large-vessel giant cell arteritis (LV-GCA)., Methods: This study included patients with LV-GCA whose treatment was discontinued between 2018 and 2023. All patients underwent PET/CT and/or MRI at the time of treatment discontinuation in clinical remission. Qualitative and quantitative PET/CT scores, by measuring standardized uptake values (SUV), and semiquantitative MRI scores of the aorta and supraaortic vessels were compared between patients who relapsed within 4 months after treatment discontinuation and those who did not., Results: Forty patients were included (median age 67.4 years, interquartile range (IQR) 60.8-74.0; 77.5 % females). Eleven patients (27.5 %) relapsed after treatment discontinuation (time to relapse 1.9 months, IQR 1.4-3.3). Patients who relapsed were comparable to those who remained in remission with respect to the presence of active vasculitis on MRI and/or PET/CT (54.5% vs. 58.6 %, p = 1.0), the number of segments with vasculitic findings on MRI (0, IQR 0.0-1.5, vs. 2, IQR 0.0-3.0, p = 0.221) or the highest SUV artery/liver ratio on PET/CT (1.5, IQR 1.4-1.6, vs. 1.3, IQR 1.2-1.6, p = 0.505). The median number of vasculitic segments on PET/CT was 2.5 (IQR 0.5-4.5) in those with vs. 0 (IQR 0.0-1.5, p = 0.085) in those without relapse, and the PET/CT scores 4.5 (IQR 0.75-8.25) vs. 0 (IQR 0.0-3.0, p = 0.172)., Conclusion: PET/CT or MRI at treatment stop did not predict relapse and may not be suited to guide treatment decisions in patients with LV-GCA in remission., Competing Interests: Declaration of competing interest AH is supported by a grant from the Swiss Foundation for Research on Muscle Diseases (FSRMM). DK received payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Abbvie, Janssen, Novartis, Pfizer, Roche, and Eli Lilly and support for attending meetings and/or travel from Janssen. DS received payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, educational events, or advisory board from Bayer, Pfizer, Bristol-Myers-Squibb, Daiichi-Sankyo, Sanofi, Philips and Bauerfeind AG. TD received payment or honoraria for lectures and advisory boards from Novartis and CSL and holds IIT grants from Novartis and Abbvie. All other authors have no competing interests., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: To update the EULAR recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV)., Methods: A systematic literature review update was performed to retrieve new evidence on ultrasound, MRI, CT and [ 18 F]-fluorodeoxyglucose positron emission tomography (FDG-PET) for diagnosis, monitoring and outcome prediction in LVV. The task force consisted of 24 physicians, health professionals and patients from 14 countries. The recommendations were updated based on evidence and expert opinion, iterating until voting indicated consensus. The level of agreement was determined by anonymous votes., Results: Three overarching principles and eight recommendations were agreed. Compared to the 2018 version, ultrasound is now recommended as first-line imaging test in all patients with suspected giant cell arteritis, and axillary arteries should be included in the standard examination. As an alternative to ultrasound, cranial and extracranial arteries can be examined by FDG-PET or MRI. For Takayasu arteritis, MRI is the preferred imaging modality; FDG-PET, CT or ultrasound are alternatives. Although imaging is not routinely recommended for follow-up, ultrasound, FDG-PET or MRI may be used for assessing vessel abnormalities in LVV patients with suspected relapse, particularly when laboratory markers of inflammation are unreliable. MR-angiography, CT-angiography or ultrasound may be used for long-term monitoring of structural damage, particularly at sites of preceding vascular inflammation., Conclusions: The 2023 EULAR recommendations provide up-to-date guidance for the role of imaging in the diagnosis and assessment of patients with LVV., Competing Interests: Competing interests: CDejaco has received consulting/speaker’s fees from Abbvie, Eli Lilly, Janssen, Novartis, Pfizer, Roche, Galapagos, Sparrow and Sanofi; grant support from AbbVie and Novartis, all unrelated to this manuscript. He is an editorial board member of ARD. SR has received research grants and/or consultancy fees From AbbVie, Eli Lilly, Galapagos, MSD, Novartis, Pfizer, Sanofi, UCB. MB: consultancy fees from AbbVie PB has received speaker fees by Janssen and project grants by Pfizer. CP has received research grants and/or consultancy fees from AbbVie, Vifor, Roche, GlaxoSmithKline and AstraZeneca, all unrelated to this manuscript. SLM reports: Consultancy on behalf of her institution for Roche/Chugai, Sanofi, AbbVie, AstraZeneca; Investigator on clinical trials for Sanofi, GSK, Sparrow; speaking/lecturing on behalf of her institution for Roche/Chugai, Vifor, Pfizer and Novartis; chief investigator on STERLING-PMR trial, funded by NIHR; patron of the charity PMRGCAuk. No personal remuneration was received for any of the above activities. Support from Roche/Chugai to attend EULAR2019 in person and from Pfizer to attend ACR Convergence 2021 virtually. SLM is supported in part by the NIHR Leeds Biomedical Research Centre. TAB reports research grants from Deutsche Forschungsgemeinschaft (DFG) and Siemens Healthineers on behalf of his Department. He has received consulting/speaker’s fees from BioTel Research, Chugai, Guerbet, Novartis, Roche, Sanofi and Siemens Healthineers. DB consultancy fees from Roche and GSKSara Brolin: Grant from Novartis. MCC has received consulting fees from GSK, SCL-Vifor, AbbVie, AstraZeneca and Janssen, and a research grant form Kiniksa Pharmaceuticals. JM-C has received consulting/speaker’s fees from Abbvie, Lilly, Janssen, Novartis, Pfizer, UCB, MSD, all unrelated to this manuscript. BD has received consultancies and educational grants from Novartis, Abbvie, Roche, Chugai, Sanofi. BDN has received consulting/speaker’s fees from Roche and Novartis all unrelated to this manuscript. EDM Research funding/consulting and conferences fees from: Abbvie, Novartis, Pfizer, Roche, Janssen, Lilly, MSD, BMS, UCB, Grunental and Sanofi. CDuftner has received consulting/speaker’s fees from Abbvie, AOP Orphan, Astra-Zeneca, Bristol-Myers-Squibb, Eli-Lilly, Janssen, Galapagos, Merck-Sharp-Dohme, Novartis, Pfizer, Roche, Sandoz, UCB, Vifor, and grant/research support from Eli-Lilly, Pfizer, UCBHaner Direskeneli is investigator in clinical trials for Abbvie and Novartis, had educational support from Pfizer, Amgene, Celltrion, UCB and Roche unrelated to this manuscript. AM has received research grants and/or consultancy fees From AbbVie, BMS, Biogen, Eli Lilly, Galapagos, Janssen, MSD, Novartis and UCB. LS has received grant support from the Swiss Society of Rheumatology, iQone and Sandoz and support for travel expenses from Sanofi; all unrelated to this manuscript. RHJAS has received independent research grants of Siemens Healtineers and WAS has received consultancy fees, honoraria and travel expenses from Abbvie, Chugai, GlaxoSmithKline, Medac, Novartis, Roche, and Sanofi and is principal investigator in trials sponsored by Abbvie, GlaxoSmithKline, Novartis and Sanofi., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To investigate the hypothesis that a history of PMR is associated with a more severe and damaging disease course in newly diagnosed GCA patients., Methods: This was a retrospective analysis of GCA patients diagnosed between December 2006 and May 2021. We compared vascular ultrasound findings (presence of vasculitis and vascular stenosis) in GCA patients with and without prior PMR., Results: Forty-nine of 311 GCA patients (15.8%) had prior PMR in a median of 30.6 (IQR 7.1-67.3) months before GCA diagnosis. Patients with prior PMR more often had large vessel vasculitis (LVV) (51.0% vs 25.0%, P < 0.001) and stenosis within the vasculitic segments (18.4% vs 3.1%, P < 0.001) on ultrasound. In multivariable analysis, prior PMR remained significantly associated with LVV (odds ratio 7.65, 95% CI: 2.72, 23.97, P < 0.001). Polymyalgic symptoms at GCA diagnosis in the patients without prior PMR were not associated with a higher prevalence of LVV (P = 0.156)., Conclusion: Patients with a diagnosis of PMR before GCA diagnosis had two times more often large vessel involvement and significant more vasculitic stenoses on ultrasound examination than patients without prior PMR. Pre-existing PMR is an independent risk factor for more extensive and advanced ultrasound findings at GCA diagnosis. The contribution of subclinical vasculitis to disease associated damage should be further studied., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Objectives: To investigate the diagnostic performance of diffusion-weighted imaging (DWI) of the superficial cranial arteries in the diagnosis of GCA., Methods: Retrospectively, 156 patients with clinically suspected GCA were included. A new 4-point ordinal DWI rating scale was developed. A post-contrast, fat-suppressed, T1-weighted 'black-blood' sequence (T1-BB) was rated for comparison. Ten arterial segments were assessed: common superficial temporal arteries, temporal and parietal branches, occipital and posterior auricular arteries bilaterally. The expert clinical diagnosis after ≥6 months of follow-up was the diagnostic reference standard. Diagnostic accuracy was evaluated for different rating methods., Results: The study cohort consisted of 87 patients with and 69 without GCA. For DWI, the area under the curve was 0.90. For a cut-off of ≥2 consecutive pathological slices, DWI showed a sensitivity of 75.9%, a specificity of 94.2% and a positive likelihood ratio of 13.09. With a cut-off of ≥3 consecutive pathological slices, sensitivity was 70.1%, specificity was 98.6% and the positive likelihood ratio was 48.38. For the T1-BB, values were 88.5%, 88.4% and 7.63, respectively. The inter-rater analysis for DWI with a cut-off of ≥2 pathological slices showed a kappa of 1.00 on the patient level and 0.85 on the arterial segment level. For the T1-BB the kappa was 0.78 and 0.79, respectively., Conclusion: DWI of the superficial cranial arteries demonstrates a good diagnostic accuracy and reliability for the diagnosis of GCA. DWI is widely available and can be used immediately in clinical practice for patients with suspected GCA., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Abstract: A 72-year-old woman presented with the fever and the pain of skull and face for 2 weeks. 18 F-FDG PET/CT equipped with semiconductor detectors revealed strong uptake not only in the temporal, cervical, subclavian arteries, and aorta, but also in the bilateral internal thoracic arteries. The diagnosis of giant cell arteritis was made. Semiconductor PET can visualize small arteries such as the internal thoracic artery. The patients with giant cell arteritis are at a high risk of ischemic heart disease, and inflammatory involvement of the internal thoracic arteries may affect the outcome of coronary artery bypass grafting., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Giant cell arteritis is a critically ischaemic disease with protean manifestations that require urgent diagnosis and treatment. European Alliance of Associations for Rheumatology (EULAR) recommendations advocate ultrasonography as the first investigation for suspected giant cell arteritis. We developed a prediction tool that sequentially combines clinical assessment, as determined by the Southend Giant Cell Arteritis Probability Score (SGCAPS), with results of quantitative ultrasonography., Methods: This prospective, multicentre, inception cohort study included consecutive patients with suspected new onset giant cell arteritis referred to fast-track clinics (seven centres in Italy, the Netherlands, Spain, and UK). Final clinical diagnosis was established at 6 months. SGCAPS and quantitative ultrasonography of temporal and axillary arteries with three scores (ie, halo count, halo score, and OMERACT GCA Score [OGUS]) were performed at diagnosis. We developed prediction models for diagnosis of giant cell arteritis by multivariable logistic regression analysis with SGCAPS and each of the three ultrasonographic scores as predicting variables. We obtained intraclass correlation coefficient for inter-rater and intra-rater reliability in a separate patient-based reliability exercise with five patients and five observers., Findings: Between Oct 1, 2019, and June 30, 2022, we recruited and followed up 229 patients (150 [66%] women and 79 [34%] men; mean age 71 years [SD 10]), of whom 84 were diagnosed with giant cell arteritis and 145 with giant cell arteritis mimics (controls) at 6 months. SGCAPS and all three ultrasonographic scores discriminated well between patients with and without giant cell arteritis. A reliability exercise showed that the inter-rater and intra-rater reliability was high for all three ultrasonographic scores. The prediction model combining SGCAPS with the halo count, which was termed HAS-GCA score, was the most accurate model, with an optimism-adjusted C statistic of 0·969 (95% CI 0·952 to 0·990). The HAS-GCA score could classify 169 (74%) of 229 patients into either the low or high probability groups, with misclassification observed in two (2%) of 105 patients in the low probability group and two (3%) of 64 of patients in the high probability group. A nomogram for easy application of the score in daily practice was created., Interpretation: A prediction tool for giant cell arteritis (the HAS-GCA score), combining SGCAPS and the halo count, reliably confirms and excludes giant cell arteritis from giant cell arteritis mimics in fast-track clinics. These findings require confirmation in an independent, multicentre study., Funding: Royal College of Physicians of Ireland, FOREUM., Competing Interests: Declaration of interests KSMvdG reports having received a speaker fee from Roche and research support from AbbVie, paid to the University Medical Center Groningen (UMCG). EC reports honoraria from GlaxoSmithKline and Chiesi. DP-P received a research contract from the Carlos III Health Institute of Spain (Rio Hortega program, reference CM20/00006), in 2021–2022; honoraria from Lilly, Novartis, AbbVie, Amgen, Merck Sharp & Dohme, S&H Medical Service Science; and support for attending meetings from Lilly, AbbVie, Pfizer. EB is an employee of the UMCG, received grants from the Dutch Arthritis Society DAS and the EU/EFPIA/Innovative Medicines Initiative 2 Joint Undertaking Immune-Image (grant number 831514) and received a speaker fee for a talk on giant cell arteritis at a post EULAR symposium organised by Mark Two Academy in the Netherlands in 2023, all were paid to the UMCG. EB is a board member of non-profit organisation ARCH (Auto-immune Research Hub) in the Netherlands. BD reports consultancy fees from Novartis, AbbVie, Sanofi. All other authors declare no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Orofacial pain is a worldwide pain problem, with many patients unable to find appropriate diagnosis and treatment. Orofacial pain includes pain arising from the odontogenic and nonodontogenic structures in the head and neck region. Dental clinicians need to have a thorough knowledge and skill to diagnose, manage, and treat patients with odontogenic pain or refer patients for treatment of nonodontogenic pain to specialists such as orofacial pain specialists, neurologists, otolaryngologists, and rheumatologists. More often, dental practitioners diagnose patients with a temporomandibular disorder (TMD), and when treatment is ineffective, term it "atypical facial pain." The first requirement for effective treatment is an accurate diagnosis. Dental clinicians must be aware of giant cell arteritis (GCA), a chronic large-vessel vasculitis, primarily affecting adults over the age of 50 years, as it frequently mimics and is misdiagnosed as TMD. GCA is associated with loss of vision, and stroke and can be a life-threatening disorder. Therefore, diagnostic testing for GCA and differential diagnosis should be common knowledge in the armamentarium of all dental clinicians. Historically, temporal artery biopsy was considered the definitive diagnostic test for GCA. Temporal artery ultrasound (TAUSG), a safe and noninvasive imaging modality, has replaced the previous diagnostic gold standard for GCA, the temporal artery biopsy, owing to its enhanced diagnostic capabilities and safety profile. The present case report describes a patient with GCA, and the role TAUSG played in the diagnosis. Case report: A 72-year-old woman presented with left-sided facial pain, jaw claudication, dysesthesia of the tongue, and episodic loss of vision of 2 years' duration. She was diagnosed with and treated for a myriad of dental conditions including endodontia and temporomandibular joint therapy with no benefit. A thorough history and physical examination, combined with serologic analysis, led to the diagnosis of GCA and TAUSG, which confirmed the diagnosis. Conclusion: This report underscores the responsibility of differential diagnosis and early recognition of GCA facilitated by TAUSG in optimizing treatment outcomes as a viable, noninvasive diagnostic tool. (Quintessence Int 2024;55:336-343; doi: 10.3290/j.qi.b4938419).

Objectives: To investigate the diagnostic accuracy of a pattern recognition approach for the evaluation of MRI scans of the head with diffusion-weighted imaging (DWI) in suspected giant cell arteritis (GCA)., Methods: Retrospectively, 156 patients with suspected GCA were included. The 'DWI-Scrolling-Artery-Sign' (DSAS) was defined as hyperintense DWI signals in the cranial subcutaneous tissue that gives the impression of a blood vessel when scrolling through a stack of images. The DSAS was rated by experts and a novice in four regions (frontotemporal and occipital, bilaterally). The temporal, occipital and posterior auricular arteries were assessed in the T1-weighted black-blood sequence (T1-BB). The diagnostic reference was the clinical diagnosis after ≥6 months of follow-up., Results: The population consisted of 87 patients with and 69 without GCA; median age was 71 years and 59% were women. The DSAS showed a sensitivity of 73.6% and specificity of 94.2% (experts) and 59.8% and 95.7% (novice), respectively. Agreement between DSAS and T1-BB was 80% for the region level (499/624; kappa(κ)=0.59) and 86.5% for the patient level (135/156; κ=0.73). Inter-reader agreement was 95% (19/20; κ=0.90) for DSAS on the patient level and 91.3% (73/80; κ=0.81) on the region level for experts. For expert versus novice, inter-reader agreement for DSAS was 87.8% on the patient level (137/156; κ=0.75) and 91.2% on the region level (569/624; κ=0.77)., Conclusions: The DSAS can be assessed in less than 1 min and has a good diagnostic accuracy and reliability for the diagnosis of GCA. The DSAS can be used immediately in clinical practice., Competing Interests: Competing interests: LS, SB, LB, FL and PS have nothing to disclose. BM: grants from Novartis; consulting fees from Novartis, Boehringer Ingelheim, Jannsen-Cilag, GSK; speaker fees from Boehringer-Ingelheim, GSK, Novartis, Otsuka, MSD; congress support from Medtalk, Pfizer, Roche, Actelion, Mepha, and MSD; patent mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143). HMB: consulting fees and speaker fees from Novartis., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ.)

Purpose: In the case of ischemic optic neuropathy (ION) or retinal artery occlusion (RAO), distinguishing arteritic from non-arteritic can limit or prevent irreversible bilateral blindness. Here, the utility of color Doppler ultrasonography (CDUS) in diagnosing giant cell arteritis (GCA) was evaluated., Methods: In this retrospective analysis, a total of 38 cases diagnosed with ION or RAO were included, that presented to our department in the years 2018 up to 2021 and underwent both CDUS and temporal artery biopsy (TAB). The evaluation is based on TAB as reference standard., Results: CDUS resulted in a sensitivity of 65.0% and a specificity of 100% (when excluding two inconclusive assessments). Therefore, when limiting TAB to only suspected cases with negative or unclear CDUS findings, the sensitivity and the specificity would remain unchanged at 100%, while reducing the need for TAB by 42.1%., Conclusion: Overall, the data suggest the implementation of a stepwise diagnostic algorithm to confirm or rule out GCA, in which the CDUS plays a key role, thus omitting the requirement for TAB in many cases., (© 2023. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

Relapses and late complications remain a concern in giant cell arteritis (GCA). Monitoring strategies are required to effectively tailor treatment and improve patients' outcomes. Current monitoring of GCA is based on clinical assessment and evaluation of traditional inflammatory markers such as C reactive protein and erythrocyte sedimentation rate; however, this approach has limited value in patients receiving interleukin (IL)-6 blocking agents. New blood biomarkers that are less dependent on the IL-6 axis such as IL-23, B cell activating factor, osteopontin and calprotectin have been explored, but none of them has yet accumulated sufficient evidence to qualify as a routine follow-up parameter. Imaging techniques, including ultrasound and 18F-fluorodeoxyglucose positron emission tomography/computed tomography, potentially offer additional insights; however, the choice of the imaging method as well as its interpretation must be investigated further. Future studies are required to investigate the outcome of patients with GCA whose treatment decisions are based on traditional plus novel (laboratory and imaging) biomarkers as compared with those undergoing conventional monitoring strategies., Competing Interests: Competing interests: AT received speaker’s fees from Novartis (unrelated to this manuscript). CD has received consulting/speaker’s fees from AbbVie, Eli Lilly and Company, Janssen, Novartis, Pfizer, Roche, Sparrow, Galapagos and Sanofi (all unrelated to this manuscript). CD is an editorial board member of ARD., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The aim of the present study was to determine the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia rheumatica (PMR) and ascertain its optimal treatment approach., Methods: Patients with PMR who fulfilled the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology Provisional Classification Criteria for PMR, did not have GCA symptoms and were routinely followed up for 2 years and were stratified into two groups, according to their ultrasound results: isolated PMR and PMR with subclinical GCA. The outcomes (relapses, glucocorticoid use and disease-modifying antirheumatic drug treatments) between groups were compared., Results: We included 150 patients with PMR (50 with subclinical GCA) with a median (IQR) follow-up of 22 (20-24) months. Overall, 47 patients (31.3 %) had a relapse, 31 (62%) in the subclinical GCA group and 16 (16%) in the isolated PMR group (p<0.001). Among patients with subclinical GCA, no differences were found in the mean (SD) prednisone starting dosage between relapsed and non-relapsed patients (32.4±15.6 vs 35.5±12.1 mg, respectively, p=0.722). Patients with subclinical GCA who relapsed had a faster prednisone dose tapering in the first 3 months compared with the non-relapsed patients, with a mean dose at the third month of 10.0±5.2 versus 15.2±7.9 mg daily (p<0.001). No differences were found between relapsing and non-relapsed patients with subclinical GCA regarding age, sex, C reactive protein and erythrocyte sedimentation rate., Conclusions: Patients with PMR and subclinical GCA had a significantly higher number of relapses during a 2-year follow-up than patients with isolated PMR. Lower starting doses and rapid glucocorticoid tapering in the first 3 months emerged as risk factors for relapse., Competing Interests: Competing interests: EDM: Research funding/consulting and conferences fees from AbbVie, Novartis, Roche, Pfizer, Janssen, Lilly, MSD, BMS, UCB, Grunenthal and Sanofi. RK, PM, EC, SC, SM, ZB, GK, PF, DK, CC and AH: No conflict of interest. CP: Research grants and/or consultancy fees from AbbVie, Vifor, Roche, GlaxoSmithKline and AstraZeneca. AT: Advisory board Novartis. IM: Speakers bureau: Roche, Novartis, UCB, Gedeon Richter and Janssen. Consultant: Roche., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)