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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.)

Background: Temporal artery biopsy (TAB) is the gold standard for the diagnosis of giant cell arteritis (GCA) but has many limitations. The Ing model, González-López model, and Weis model are tools to estimate a patient's likelihood of GCA. This study investigates the utility of these prediction models in triaging patients referred for TAB., Methods: This study is a retrospective examination of patients who underwent TAB by a neuro-ophthalmologist over a 5-year period. Data collected through chart review were inputted into prediction models to evaluate GCA risk and compared with TAB results and clinical diagnosis. Cut-off values for 100% sensitivity and specificity for TAB result were used to determine whether TAB could be avoided where there was preoperative certainty of the result., Results: Among 155 eligible patients, mean age was 73 years, and 78.1% were female. TAB was negative in 103 patients (66.5%) and positive in 42 patients (27.1%). Twenty-three patients (22.3%) were diagnosed clinically and treated for biopsy-negative GCA. The Ing model had no positive biopsies below 10.59% and no negative biopsies above 68.44%. The González-López model had no positive biopsies below 0.27% and no negative biopsies above 98.08%. The Weis model had no positive biopsies with a score less than zero., Conclusion: Forty-one biopsies (28.9%) could have been avoided using the Ing model, 9 (6.34%) using the González-López model, and 28 (19.7%) using the Weis model. The findings suggest that the Ing and Weis models are useful screening tools for GCA with the potential to improve the effective use of health care resources., Competing Interests: Footnotes and Disclosure The authors have no proprietary or commercial interest in any materials discussed in this article., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Giant cell arteritis (GCA), the most common primary vasculitis in adults, is a granulomatous systemic vasculitis usually affecting the aorta and its major branches, particularly the carotid and vertebral arteries. Although remission can be achieved in most patients with GCA using high-dose glucocorticoids (GC), relapses are frequent, occurring in >40% of GC-only treated patients, mostly during the first two years after diagnosis. Relapsing courses lead to high GC exposure, increasing the risk of treatment-related adverse effects. Although tocilizumab is an efficacious GC-sparing therapy that allows increased sustained remission and reduced cumulative GC doses, relapses are common after drug discontinuation. This narrative review examines the most relevant features of relapses in GCA, including its definition, classification, frequency, clinical, laboratory, and imaging characteristics, chronology, probable pathophysiology, and predictive factors. In addition, we discuss treatment options for relapsing patients and the effect of relapses on patient outcomes., 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., (Copyright © 2024. Published by Elsevier B.V.)

Introduction: The management of giant cell arteritis (GCA) has evolved with the arrival of tocilizumab (TCZ) and the use of PET/CT. Our objective is to describe the characteristics and followup of patients with recent diagnosis of GCA in current care., Patients and Methods: The NEWTON cohort is a monocentric retrospective cohort based on data collected from 60 GCA patients diagnosed between 2017 and 2022 according to the ACR/EULAR 2022 criteria., Results: The median age at diagnosis was 73 [68.75; 81] years old. At diagnosis, the main manifestations were unusual temporal headaches in 48 (80 %) and an inflammatory syndrome in 50 (83 %) patients. Temporal artery biopsy confirmed the diagnosis in 49/58 (84 %) patients. Doppler of the temporal arteries found a halo in 12/23 (52 %) patients. The PET/CT found hypermetabolism in 19/43 (44 %) patients. Prednisone was stopped in 17.5 [12.75; 24.25] months. During follow-up, 22 (37 %) patients received TCZ. At least one complication of corticosteroid therapy was observed in 22 (37 %) patients. After a median follow-up of 24 [12; 42] months, 25 (42 %) patients relapsed. At the end of the follow-up, 29 (48.3 %) patients were weaned from corticosteroid therapy and 15 (25 %) were on TCZ., Conclusion: Despite the increasing use of TCZ in the therapeutic arsenal and of the PET/CT in the imaging tools of GCA patients, relapses and complications of corticosteroid therapy remain frequent, observed in more than a third of patients., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common conditions in older adults. Their clinical connection has been recognized over time, with many patients experiencing both conditions separately, simultaneously or in temporal sequence to each other. Early GCA detection is essential to prevent vascular damage, but identifying subclinical GCA in PMR patients remains a challenge and routine screening is not standard practice. Subclinical GCA prevalence in newly diagnosed PMR patients ranges from 23 to 29%, depending on the screening method. Vessel wall imaging and temporal artery biopsy can detect subclinical GCA. Epidemiology and trigger factors show similarities between the two conditions, but PMR is more common than GCA. Genetic and pathogenesis studies reveal shared inflammatory mechanisms involving dendritic cells, pro-inflammatory macrophages, and an IL-6 signature. However, the inflammatory infiltrates differ, with extensive T cell infiltrates seen in GCA while PMR shows an incomplete profile of T cell and macrophage-derived cytokines. Glucocorticoid treatment is effective for both conditions, but the steroid requirements vary. PMR overall mortality might be similar to the general population, while GCA patients with aortic inflammatory aneurysms face increased mortality risk. The GCA-PMR association warrants further research. Considering their kinship, recently the term GCA-PMR Spectrum Disease (GPSD) has been proposed., 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., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: To evaluate the difference in temporal artery biopsy length before and after formalin fixation and identify any correlations with pathologic diagnosis., Design: Prospective case series., Participants: Patients undergoing temporal artery biopsy between June 2020 and October 2021., Methods: The pre- and postfixation biopsy lengths were compared. The primary outcome was the difference in temporal artery length as measured before fixation by the surgeon versus the postfixation measurement by the pathologist., Results: Forty-seven consecutive biopsies in 46 patients were included. One patient had a repeat biopsy. Mean age was 75.3 ± 8.4 years (range, 49-94 years); 74% of patients (34 of 46 patients) were female. Mean prefixation biopsy length was 2.36 ± 0.58 cm (range, 1.1-4.5 cm). Mean postfixation biopsy length was 2.09 ± 0.59 cm (range, 0.6-3.8 cm). Mean difference (postfixation shrinkage) was 0.27 ± 0.24 cm (p = 0.0298), and 36% of biopsies (17 of 47 biopsies) were positive. There was no significant difference in prefixation temporal artery biopsy length (p = 0.38) or postfixation shrinkage (p = 0.24) between positive and negative biopsies. In a univariate analysis, elevated erythrocyte sedimentation rate was 31.3 mm/h (range, 4-88 mm/h) in negative biopsies versus 54.5 mm/h (range, 29-98 mm/h) in positive biopsies (p = 0.01), C-reactive protein was 17.4 mg/L (range, 0.2-145 mg/L) in negative biopsies versus 78.56 mg/L (range, 5-244.4 mg/L) in positive biopsies (p = 0.003), and platelets were 254.9 × 10 9 /L (range, 134-570 × 10 9 /L) in negative biopsies versus 393.8 × 10 9 /L (range, 210-593 × 10 9 /L) in positive biopsies (p < 0.001), all associated with a positive pathologic diagnosis., Conclusions: The average temporal artery biopsy was approximately 0.27 cm shorter on pathologic reports compared with before fixation measurements. Surgeons should account for this shrinkage with a buffer of at least 0.3 cm, aiming for excision of at least 2.3 cm, if they desire a postfixation size of 2 cm., (Copyright © 2022 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Polymyalgia rheumatica is an inflammatory disease producing pain and stiffness, mainly in the shoulders and pelvic girdle, in people older than 50 years. Elevation of acute phase reactants is common due to the inflammatory nature of the disease. Since there are no specific diagnostic tests, diagnosis requires the exclusion of other diseases with similar presentations. Imaging has helped to identify the pathological substrate of polymyalgia rheumatica and it is increasingly used to support clinical diagnosis or to detect coexistent giant cell arteritis. Although polymyalgia rheumatica does not clearly impair survival or organ function, it can have a detrimental effect on quality of life. Glucocorticoids at 12·5-25·0 mg prednisone per day are effective in inducing remission in most individuals but, when tapered, relapses occur in 40-60% of those affected and side-effects are common. Assessment of disease activity can be difficult because pain related to common comorbidities such as osteoarthritis and tendinopathies, can return when glucocorticoids are reduced, and acute phase reactants are increased less during flares in individuals undergoing treatment or might increase for other reasons. The role of imaging in assessing disease activity is not yet completely defined. In the search for more efficient and safer therapies, tocilizumab and sarilumab have shown efficacy in randomised controlled trials and additional targeted therapies are emerging. However, judicious risk-benefit balance is essential in applying therapeutic innovations to people with polymyalgia rheumatica., Competing Interests: Declaration of interests GE-F reports consulting fees from Janssen, GSK, and CSL Vifor, and meeting travel support from Boehringer Ingelheim. CD reports grant support from AbbVie, Novartis, and Celgene; consulting fees, speaker fees, or both honoraria from AbbVie, Roche, Sanofi, Galapagos, Lilly, Janssen, Novartis, and Sparrow; participation in advisory boards for Roche, Sanofi, AbbVie, Janssen, Novartis, Eli Lilly, Galapagos, and Sparrow; meeting travel support from AbbVie, Roche, Sanofi, Galapagos, Eli Lilly, Janssen, and Novartis; and royalties from Oxford University Press and SAGE journals. CD reports participation in the EULAR congress committee. SLM received a research grant from CSL Vifor and from the National Institute of Health Research; consulting fees from AbbVie, AstraZeneca, Roche/Chugai, and Sanofi; lecturing fees from Pfizer, UCB, and CSL Vifor; and meeting travel support from Roche/Chugai and Pfizer. SLM is a patron of the charity Polymyalgia Rheumatica and Giant Cell Arteritis UK. CS reports consulting fees from AbbVie, Boehringer Ingelheim, Roche, Lilly, Galapagos, Pfizer, Novartis, and Amgen, and royalties from UpToDate. ELM reports a research grant from AbbVie; consulting fees, lecturing fees, or both from Boehringer Ingelheim and Novartis; royalties from UpToDate; and speaker fees from Practice Point Communication. ELM reports participation in advisory boards for National Institutes of Health and Horizon Therapeutics. MCC reports consulting fees from GSK, AbbVie, CSL Vifor, Janssen, and AstraZeneca; royalties from UpToDate; and a research grant and meeting travel support from Kiniksa Pharmaceuticals. MCC reports participation in advisory boards for GSK, CSL Vifor, and AstraZeneca., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: To examine the performance of the new 2022 American College of Rheumatology (ACR)/EULAR classification criteria for giant cell arteritis (GCA) in routine clinical care, compared with the 1990 ACR GCA classification criteria., Methods: The fulfilment of 2022 ACR/EULAR and 1990 ACR criteria was tested in our real-life cohort of GCA patients with proven vasculitis by temporal artery biopsy or imaging (a necessary pre-requisite to apply the new criteria is the presence of a confirmed diagnosis of medium- or large-vessel vasculitis). The performance of classification criteria was evaluated in all patients with GCA across different subsets of the disease. Patients with GCA were compared with unselected controls with suspected GCA., Results: A total of 136 patients with proven GCA were identified. The new criteria had a sensitivity of 92.6% and a specificity of 85.2%. According to the clinical phenotypes, the sensitivity was 98.8% in cranial GCA, 92% in extracranial large vessel (LV) GCA and 75% in occult systemic GCA. These data are much better than those observed with the 1990 ACR classification criteria, which showed a sensitivity of 66.1% and a specificity of 85.1% for the total sample, with a sensitivity of 89.1% in cranial GCA, 24% in extracranial LV-GCA and 35.7% in occult systemic GCA. Ten (7.4%) patients in our cohort did not fulfil either of the criteria sets (8 with occult systemic GCA and 2 with extracranial LV-GCA). The sensitivity of the new criteria in patients with occult systemic and extracranial LV-GCA could be greatly improved assigning more weight (3 points) to some imaging findings (axillary involvement and FDG-PET activity throughout the aorta)., Conclusion: Our study confirms that the new classification criteria are more sensitive in real-life settings than the old ACR criteria across all clinical phenotypes., Competing Interests: Declaration of Competing Interest The authors declare that there are no competing interests., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Introduction: Coronaritis is a rare but serious complication of giant-cell arteritis (GCA), with an estimated prevalence of less than 1%, however difficult to establish, and of early onset., Methods: We describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication., Results: The first patient presented with stable angina on common trunk coronaritis with ostial stenosis. Corticosteroid combined with tocilizumab from the outset resulted in improvement. Angioplasty was performed at 6months with good outcome. The second patient presented with asymptomatic tritruncular ostial coronaritis. Corticosteroid allowed clinic-biological improvement of GCA. Two years later, he presented relapse with an acute coronary syndrome, with favorable evolution after angioplasty, increase of corticosteroids and addition of tocilizumab., Conclusion: Patients presented were successfully treated with corticosteroids combined with tocilizumab and angioplasty of their coronary stenoses. Efficacy of tocilizumab in GCA has not been evaluated especially on coronaritis due to the rarity of this complication. Our experience and the cases reported in the literature suggest good results of angioplasty in this indication. Studies with long-term follow-up will be necessary to evaluate the risk of restenosis., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Purpose: Giant cell arteritis (GCA) involving ophthalmic circulation often manifests as anterior ischemic optic neuropathy (AAION), presenting with severe vision loss and pallid optic disc edema. Non-arteritic anterior ischemic optic neuropathy (NAION) classically presents with segmental optic disc edema and corresponding altitudinal visual field defect (VFD) with small cup-to-disc ratio in the fellow eye. Differentiating these two entities is critical as GCA requires immediate treatment to prevent vision loss in the fellow eye. This study investigated how often GCA mimics NAION at presentation., Methods: Retrospective chart review of patients with temporal artery biopsy (TAB) positive GCA with ocular manifestations seen at a tertiary neuro-ophthalmology practice between 2015 and 2020. Patients presenting with segmental non-pallid optic disc swelling and corresponding altitudinal VFD mimicking NAION were identified., Results: The clinical presentation of 7.1% (3/42) of patients with TAB-positive GCA mimicked NAION. Two of three patients had cup-to-disc ratio of <0.3 in the fellow eye. Two patients were women, mean age was 67.3 ± 6.5 years, and mean presenting visual acuity was 0.45 ± 0.48 LogMAR. Two patients had a normal temporal artery ultrasound. Two of three patients had at least one systemic symptom of GCA at presentation and all had elevation of one or both inflammatory markers., Conclusions: There should be high index of suspicion for GCA, even in patients highly suspected to have NAION. Inflammatory markers must be checked in every patient with presumed NAION and TAB performed if one or both are elevated to avoid missing GCA., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Introduction: SARS-CoV-2 infection and COVID-19 vaccines might have increased the incidence of giant-cell arteritis (GCA) and the risk of associated stroke in Spain., Methods: Retrospective nation-wide observational analysis of all adults hospitalized with GCA in Spain during 5 years (Jan-2016 and Dec-2021). The incidence and proportion of admissions with or because of GCA and GCA-associated stroke were compared between pre-pandemic (2016-2019) and pandemic (2020 and 2021) years. Sensitivity analyses were conducted for the different COVID-19 waves and vaccine timing schedules., Results: A total of 17,268 hospital admissions in patients diagnosed with GCA were identified. During 2020 there were 79.3 and 8.1 per 100,000 admissions of GCA and GCA-associated stroke, respectively. During 2021 these figures were 80.8 and 7.7 per 100,00 admissions, respectively. As comparison, yearly admissions due to GCA and GCA-associated stroke were 72.4 and 5.7 per 100,00, respectively, during the pre-pandemic period (p < 0.05). Coincident with the third wave of COVID-19 (and first vaccine dosing), the rate of GCA-associated stroke admissions increased significantly (from 6.7 to 12%; p < 0.001). Likewise, there was an increase in GCA-associated stroke (6.6% vs 4.1%, p = 0.016) coincident with the third dose vaccination (booster) in patients older than 70 at the end of 2021. In multivariate analysis, only patients admitted during the third COVID-19 wave (and first vaccine dosing) (OR = 1.89, 95% CI 1.22-2.93), and during the third vaccination dosing in patients older than 70 (booster) (OR = 1.66, CI 1.11-2.49), presented a higher GCA-associated stroke risk than the same months of previous years after adjustment by age, sex, classical cardiovascular risk factors and COVID-19 diagnosis., Conclusions: The COVID-19 pandemic led to an increased incidence of GCA during 2020 and 2021. Moreover, the risk of associated stroke significantly risen accompanying times of COVID-19 vaccine dosing, hypothetically linked to an increased thrombotic risk of mRNA-SARS-CoV-2 vaccines. Hence, forthcoming vaccine policies and indications must weigh the risk of severe COVID-19 with the risk of flare or stroke in patients with GCA., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Victor Moreno-Torres reports financial support was provided by Academia Médico-Quirúrgica Española. Guillermo Ruiz-Irastorza reports financial support was provided by Department of Education of the Basque Government., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Giant cell arteritis (GCA) is a potentially devastating disease that may require treatment with high-dose steroids. Traditionally, diagnosis requires patients to meet at least 3 of 5 clinical criteria, one of which is a positive temporal artery biopsy (TAB). Vascular surgeons are often asked to perform TAB though it is not necessarily required for diagnosis or management. This study aimed to determine if TAB results altered management of patients with a concern for GCA by changing steroid use postoperatively in our health care system., Methods: A retrospective review at a single-center tertiary care hospital was performed between 2007 and 2018. The inclusion criteria were patients greater than 18 years old with complete steroid treatment records who underwent a temporal artery biopsy due to concern for GCA. Steroid use and duration of treatment both pre- and post-operative were collected and analyzed., Results: Eighty-three of 117 cases reviewed met inclusion criteria. Ninety-one percent (76) of patients had a negative biopsy. Twenty-nine percent (23) of negative biopsies met criteria for GCA prior to biopsy. Of those with a negative biopsy, steroids were continued in 68% (52) of patients after 30 days, 49% (37) after 90 days and 45% (34) after 180 days. Steroids were never started in 11% (6). One patient with a positive biopsy was discontinued on steroids due to intolerance. There was no statistically significant difference in duration of steroids between those with a positive and negative biopsy (average 610 and 787 days respectively; P = 0.682). Average follow up was 33 months., Discussion: The duration of steroid use for patients with concern for GCA was not found to be altered by the performance of a TAB at our institution. Given the extremely low yield and absence of impact on steroid duration, TAB is not a useful diagnostic test at our institution. Similar reviews are recommended to determine the utility of TAB at other institutions that may differ in patient population or prescribing practices., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Infections are associated with morbimortality of patients with giant cell arteritis (GCA). The aim of this work was twofold: the identification of factors predisposing to the risk of infection and the description of patients hospitalized with an infection occurring during the treatment period of CAG., Methods: A monocentric retrospective study was conducted in GCA patients, comparing patients hospitalized for infection with patients without infection. The analysis included 21/144 (14.6%) patients with 26 infections (cases) and 42 control matched on sex, age, and diagnosis of GCA., Results: Both groups were similar except for a higher frequency of seritis in cases (15% vs. 0%, p=0.03). Relapses of GCA were less common in cases (23.8% vs 50.0%, p=0.041). Hypogammaglobulinemia was present during infection. More than half of the infections (53.8%) occurred in the first year of follow-up with an average dose of 15mg/day of corticosteroids. Infections were mainly pulmonary (46.2%) and cutaneous (26.9%)., Conclusion: Factors associated with infectious risk were identified. This preliminary monocentric work will continue with a national multicentre study., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Imaging is increasingly being used to guide clinical decision-making in patients with giant cell arteritis (GCA). While ultrasound has been rapidly adopted in fast-track clinics worldwide as an alternative to temporal artery biopsy for the diagnosis of cranial disease, whole-body PET/CT is emerging as a potential gold standard test for establishing large vessel involvement. However, many unanswered questions remain about the optimal approach to imaging in GCA. For example, it is uncertain how best to monitor disease activity, given there is frequent discordance between imaging findings and conventional disease activity measures, and imaging changes typically fail to resolve completely with treatment. This chapter addresses the current body of evidence for the use of imaging modalities in GCA across the spectrum of diagnosis, monitoring disease activity, and long-term surveillance for structural changes of aortic dilatation and aneurysm formation and provides suggestions for future research directions., Competing Interests: Declaration of competing interest Author CEO declares advisory board for AbbVie; speaking honoraria from AbbVie, Novartis, and Janssen and Roche; co-investigator on STERLING-PMR trial, funded by NIHR-NHMRC; investigator on AbbVie and Novartis clinical trials. Author MY declares advisory board for BioGen; co-investigator on STERLING-PMR trial, funded by NIHR-NHMRC; financial support from AbbVie, and UCB to attend international conferences, including the BSR and EULAR. Author DFL co-investigator on STERLING-PMR trial, funded by NIHR-NHMRC. Author AMP declares no conflict of interest. Author HIK declares speaking honoraria from AbbVie, and Roche and Jansen; co-investigator on STERLING-PMR trial, funded by NIHR-NHMRC; investigator on AbbVie, Sanofi, and Novartis clinical trials; educational support from Pfizer. Author CLH declares co-investigator on STERLING-PMR trial, funded by NIHR-NHMRC; grant support from Vifor. Author SLM declares consultancy on behalf of her institution for Roche/Chugai, Sanofi, AbbVie and AstraZeneca; investigator on clinical trials for Sanofi, GSK, and Sparrow; speaking/lecturing on behalf of her institution for Roche/Chugai, Vifor, Pfizer, UCB, and Novartis; chief investigator on STERLING-PMR trial, funded by NIHR-NHMRC; 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. The views expressed in this article are those of the authors and not necessarily those of the NIHR, the NIHR Leeds Biomedical Research Centre, the National Health Service, or the UK Department of Health and Social Care., (Crown Copyright © 2023. Published by Elsevier Ltd. All rights reserved.)

Rheumatologists are increasingly using vascular ultrasound. Several guidelines now recommend ultrasound as the first diagnostic modality in giant cell arteritis (GCA). The German curriculum for rheumatology training has recently included ultrasound for the acute diagnosis of vasculitis. Recent studies have shown that ultrasound of temporal, axillary, subclavian, and vertebral arteries has sensitivities and specificities of >90%. Vascular ultrasound detects subclinical GCA in approximately 20% of patients with "pure" polymyalgia rheumatica. GCA fast-track clinics might regularly include these patients. A new score based on the intima-media thickness of the temporal and axillary arteries allows the monitoring of structural changes with treatment. The score decreases faster for the temporal arteries than it does for the axillary arteries. Measuring the diameter of the ascending aorta and the aortic arch might become a fast and cost-effective tool for the long-term monitoring of aortic aneurysms in extracranial GCA. Vascular ultrasound also has a role for Takayasu arteritis, thrombosis, Behçet's syndrome, and Raynaud's phenomenon., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Objectives: Permanent visual impairment is a major complication of giant cell arteritis (GCA). We investigated the added value of color Doppler imaging (CDI) of the central retinal artery (CRA) in patients with suspected GCA for early risk evaluation before temporal artery biopsy (TAB) results become available., Methods: We conducted a non-interventional observational study of 30 consecutive patients hospitalized for suspected GCA, including a comprehensive analysis of clinical, laboratory, imaging, CDI and pathology data. GCA was diagnosed or excluded (GCA+, GCA-, respectively) according to American College of Rheumatology (ACR) criteria and TAB findings. Three patients not meeting ACR criteria were excluded secondarily. The GCA- group contained ten patients, and the GCA+ group contained 17 patients, including eight with unilateral, transient or permanent clinical visual impairment (CVI)., Results: Mean blood flow velocity (mBFV) in the CRA was impaired in the affected eyes of GCA + CVI+ patients (1.9 ± 0.9 cm.s -1 , p < 0.001) relative to controls (4.1 ± 1.0 cm.s -1 ), GCA- patients (3.6 ± 0.7 cm.s -1 ) and GCA + CVI- patients (3.8 ± 0.8 cm.s -1 ). The mBFVs of the CRA was similar for affected and fellow eyes (right or left). CRA mBFV measurements effectively differentiated between patients with and without CVI (ROC-curve analysis, AUC = 0.925 [95%CI: 0.700 to 0.996], p < 0.0001, 88% sensitivity, 89% specificity, and cutoff of ≤2.7 cm.s -1 for affected eyes; 75% sensitivity, 100% specificity and cutoff of ≤2.2 cm.s -1 for fellow eyes)., Conclusion: CDI facilities the early detection of visual ischemia risk in GCA+ patients, justifying urgent high-dose corticosteroid administration to save at least the fellow eye before pathology results become available., 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., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Giant cell arteritis is a common vasculitis in patients over the age of 50 years old. If not promptly recognized and aggressively treated with high-dose glucocorticoids, ischemia resulting in permanent vision loss or stroke can occur. Yet, the treatment with high-dose glucocorticoids over a long period of time can be problematic in this particular patient population given their age and associated comorbidities. Temporal artery biopsies (TAB) are an important diagnostic tool to evaluate patients with suspected giant cell arteritis. Herein, we explore indications for TAB and practical points in obtaining a TAB based on available evidence. We review the surgical procedure itself and associated complications. Lastly, we examine common pathological findings and considerations of alternative diagnoses., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Introduction: Hearing loss is a rare manifestation in giant cell arteritis. The different types of deafness are possible with a predominance of sensorineural deafness., Case Report: We report a 75-year-old woman who presented with typical manifestations of giant cell arteritis associated concomitantly with the occurrence of bilateral mixed hearing loss confirmed on the audiogram. Corticosteroids allowed a rapidly favorable clinical and biological outcome. The follow-up audiogram at 3 months was markedly improved and showed a decrease in sensorineural hearing loss and disappearance of conductive hearing loss., Conclusion: Any rapid onset deafness in an inflammatory context in the elderly should lead to a search for giant cell arteritis. The diagnosis can be difficult in the absence of other typical manifestations, especially since the biopsy of the temporal artery most often comes back negative. Corticosteroids are usually effective., (Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

For decades, aside from prednisone and the occasional use of immune suppressive drugs such as methotrexate, there was little to offer patients with polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). However, there is a great interest in various steroid sparing treatments in both these conditions. This paper aims to provide an overview of our current knowledge of PMR and GCA, examining their similarities and distinctions in terms of clinical presentation, diagnosis, and treatment, with emphasis placed on reviewing recent and ongoing research efforts on emerging treatment. Multiple recent and ongoing clinical trials are demonstrating new therapeutics that will provide benefit and contribute to the evolution of clinical guidelines and standard of care for patients with GCA and/or PMR., Competing Interests: Declaration of competing interest Nothing to declare., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Objective: A surgical temporal artery biopsy (TAB) is the gold standard for diagnosis of giant cell arteritis (GCA). The necessity of performing a bilateral biopsy remains under debate. The primary objective of this study was to assess the rate of discordance between pathology results in patients who underwent bilateral TAB for suspected GCA., Methods: We performed a retrospective review of patients who underwent bilateral TAB for the diagnosis of GCA between 2011 and 2020. The primary end point was the rate of discordance between specimens for patients with pathology positive GCA. Secondary end points included assessments of the sensitivity of preoperative temporal artery duplex and the effects of specimen length and specialty of referring provider on the diagnostic yield of the biopsy., Results: During the study period, 310 patients underwent bilateral TAB for the diagnosis of GCA. These patients were primarily female (73.9%), elderly (mean age, 70.8 years), and Caucasian (95.8%). Preoperative symptoms for patients were typically bilateral (59%) and included headache (81%), vision changes (45.2%), and temporal tenderness (32.6%). Most patients (85.2%) were on preoperative steroid therapy at the time of surgical biopsy with a mean preoperative duration of steroid therapy of 15.1 days. Overall, 91 patients (29.4%) had a positive pathologic diagnosis after bilateral TAB. Of these patients, 11 had a positive pathology result in only a single specimen, resulting in a discordance rate of 12.1%. Preoperative temporal artery duplex demonstrated a low sensitivity (27.3%) for identifying patients with pathologic positive disease. There were no significant differences between the pathology-positive and -negative patients in terms of mean surgical specimen length (1.67 cm vs 1.64 cm; P = .67) or the specialty of the referring provider (P = .73)., Conclusions: At our institution, we observed a 12.1% discordance rate between pathology results in patients who underwent bilateral TAB for diagnosis of GCA. A preoperative temporal artery duplex provided little value in identifying patients with biopsy-proven GCA., (Copyright © 2022 Society for Vascular Surgery. All rights reserved.)

Objective: To identify the clinical, laboratory, and histopathological features that may predict the diagnosis of giant cell arteritis (GCA)., Methods: A retrospective chart review was performed on patients who underwent temporal artery biopsy (TAB) between January 1, 2011 and March 31, 2019. Patient demographics, clinical characteristics, laboratory features, histopathological features, and biopsy results were collected. GCA status was determined by a neuro-ophthalmologist (OOA). Stepwise logistic regression analysis was performed to identify features that predict GCA status., Results: Of 101 patients who underwent TAB, 31 (31%) were diagnosed with GCA. Age was found to be statistically significant for the diagnosis of GCA (P = 0.009), with an average age of 74.4 years ( ± 8.1) in those with GCA vs. 68.9 years ( ± 10.0) in those without. The incidence of transient vision loss was higher in GCA than non-GCA patients (P = 0.005). Anterior arteritic ischemic optic neuropathy (n = 3), ophthalmic artery occlusion (n = 2), and posterior ischemic optic neuropathy (n = 1) were seen only in the GCA group. Of the 31 GCA patients, 15 had active GCA (48%), 3 (10%) had healed temporal arteritis (HTA), 8 (26%) had suggested HTA, and 5 (16%) had false negative biopsies. Of the 70 non-GCA patients, 63 (90%) had negative biopsies, 2 (3%) had HTA, and 5 (7%) had suggested HTA. Histopathological analysis revealed that CD68 staining had a sensitivity of 69% and specificity of 86%. Both presence of multinucleated giant cells (MNGC) and transmural inflammation had 100% specificity; however, sensitivity was ≤ 50%. In patients with negative TABs and complete risk factor data available (n = 66), the odds of GCA increased 2.16-fold every 5 years of age, and 1.08-fold every mg/day of oral steroid use. A biopsy result of HTA had an odds ratio of 84.7 and suggested HTA of 49.2 against a negative TAB for diagnosis of GCA., Conclusion: Age at time of biopsy, HTA, and suggested HTA are predictive for the diagnosis of GCA. Transient vision loss is more commonly seen in GCA, and anterior arteritic ischemic optic neuropathy, ophthalmic artery occlusion, and posterior ischemic optic neuropathy are important ophthalmic manifestations of GCA. CD68 staining is more sensitive but less specific for diagnosing GCA in comparison to other histopathologic findings such as presence of MNGC and transmural inflammation. Further work is recommended to investigate the importance of the specific histopathologic finding of CD68 staining in the diagnosis of GCA., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Q fever is a worldwide spread zoonotic disease, caused by the gram-negative intracellular bacillus Coxiella burnetii. Apart from its most common manifestations, Q fever has been reported to occasionally mimic autoimmune diseases. We herein present a case of acute Q fever in a 69-year-old man, manifesting as prolonged fever with pneumonitis, in whom biopsy of the temporal artery revealed giant cell arteritis. Moreover, PCR testing of the biopsy specimen was positive for Coxiella burnetii, thus further supporting the possibly infectious etiology of some cases of biopsy proven giant cell arteritis, with implications for treatment., (Copyright © 2022 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are considered 2 diseases on the same spectrum due to their many underlying similarities. In recent years, both diseases have witnessed both diagnostic and treatment advances, which shaped the way we manage them. In this article, the authors focus on different diagnostic modalities in GCA as well as the presence of different clinical phenotypes and the role of screening for aortic involvement. The authors also discuss traditional treatments and the role of evolving steroid-sparing agents in the management of both GCA and PMR., Competing Interests: Disclosure The authors have nothing to disclose that is relevant to this article., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: Genital vasculitis are uncommon. They may be localized or be a manifestation of a systemic vasculitis. We report a patient with a giant cell arteritis (GCA) involving uterine arteries and a literature review on genital vasculitis., Case Report: A 65-year-old woman was referred to a gynecologist for a cervical intraepithelial neoplasia (CIN) associated with an ovarian mass. An unexpected diagnosis of GCA involving small to medium sized uterine arteries was made through the anatomopathological analysis while the patient was asymptomatic. Two weeks later, she presented typical cranial symptoms of giant cell arteritis (GCA). PET-scanner confirmed the diagnosis of GCA with an involvement of the ascending aorta, and the axillary and the subclavian arteries., Conclusion: Gynecologic vasculitis are rare and usually an asymptomatic manifestations of GCA., (Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Objective: The relapse rate of giant cell arteritis (GCA) is around 48%. Major relapse of GCA is defined by the European League Against Rheumatism as severe ischemic or aortic (stenosis, aneurysm, or aortic dissection) disease of GCA. The objective of the present study was to determine the prevalence and incidence, as well as the spectrum of major relapse in GCA using published data., Methods: The MEDLINE and Cochrane databases were searched up to March 2020. Studies that included patients with newly diagnosed or relapsed GCA receiving glucocorticoids (GC) alone and/or GC-sparing therapy, detailing the number of relapsing patients and the characteristics of relapses were included. The prevalence and incidence of major relapse were pooled using a random-effects model., Results: Twenty-six studies (including eight randomised controlled trials) involving 2754 patients with GCA were included. The prevalence and incidence of major relapse in this population was 3.3% (95%CI [1.7;5.6]; I 2  = 86%) and 14.5/100 patient-years (95%CI [5.2;27.2]; I 2  = 90%). The clinical manifestations were jaw claudication (44.3%), ophthalmological involvement (32.7%), peripheral limb ischemia (12.5%), aortic (7.7%), and neurological involvements (4.8%). In the meta-regression analysis, the duration of follow-up was negatively associated with the incidence of major relapse (Beta = -0.015, 95%CI [-0.026; -0.0042]; p = 0.0063). The incidence of major relapse was significantly higher in prospective studies (55.2/100 person-years, 95%CI [15.3;114.3] than in retrospective studies (4.1/100 patient-years, 95%CI[1.1;8.4]; p interaction  = 0.000.2)., Conclusion: This study found that there was heterogeneity among studies, and this is partially related to study design. Jaw claudication was frequent and increases the prevalence and incidence of relapses major., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction: Headache, visual disturbances and shoulder and hip girdle pain are frequent symptoms of consultation and the diagnosis of giant cell arteritis (GCA) can be evoked in these situations. However, GCA is a rare disease, and the management modalities of a clinical suspicion of GCA are poorly described, which warranted this study., Methods: This is a survey evaluating the management of a suspected case of GCA. The questionnaires were sent to general practitioners (GPs), members of the French Rheumatology Society (SFR) and the French National Society of Internal Medicine (SNFMI) RESULTS: One thousand four hundred and fifty two physicians responded to the survey, including 967 GPs (66.6 %) and 485 other specialists (33.4 %). GPs immediately referred the patient to the emergency room in 42 % of cases, and to a specialist colleague in 72 % of cases in the presence of visual symptoms. GPs and other specialists reported performing temporal artery biopsy (TAB) respectively in 46.7 % and 69.7 % of cases (P<0.05). GPs and other specialists reported using diagnostic imaging in 7.4 % and 16.2 % of cases, respectively (P<0.05). Temporal artery ultrasound was the most used diagnostic imaging. The average prednisone equivalent dose prescribed as initial treatment was 1mg/kg/day for GPs and 0.7mg/kg/day for other specialists (P<0.05)., Conclusion: Some suspected GCA patients would be managed by their GPs. Imaging was little used for the diagnosis of GCA and TAB remained the preferred diagnostic examination. The initial prednisone equivalent dose varied between GP and other specialists., (Copyright © 2021. Published by Elsevier Masson SAS.)

Introduction: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are inflammatory rheumatic diseases common in people over the age of 50 years. Seasonal influenza vaccination (IV) is strongly recommended in this population, among whom it is considered to be effective and well tolerated. IV-induced GCA or PMR are thought to be exceptional., Patients and Methods: We retrieved all post-IV cases from an inception cohort of patients with newly diagnosed GCA. We also included two patients with post-IV PMR and reviewed all published reports of post-IV GCA or PMR, with selection of cases demonstrating disease onset within 1 month following IV. We compared the results of HLA-DRB1 typing, performed in seven patients with post-IV GCA or PMR, with those of 11 GCA patients with familial aggregation and 16 randomly selected GCA patients without a reported trigger., Results: Of 358 GCA recruited since 2002, 10 (2.8%) qualified for post-IV GCA, of whom two also showed familial aggregation. Thirty-two patients (19 with GCA and 13 with PMR) including our patients were reviewed; their mean age was 71.8 ± 7.4 years and the M/F ratio was 0.8. Six patients (19%) had a history of PMR. Patients with post-IV GCA/PMR had the DRB1*13:01 haplotype more frequently compared to those with familial GCA (5/7 vs. 2/11, p = 0.048) or with GCA without a known trigger (3/16, p = 0.026). Post-IV PMR generally appeared self-limited, whereas post-IV GCA often displayed a more protracted course (chronic relapsing disease in one-third of the patients)., Conclusion: Post-IV onset of GCA/PMR is not an exceptional occurrence and may be part of the spectrum of the autoimmune syndrome induced by adjuvants (ASIA). IV can trigger GCA or PMR, especially in persons at higher spontaneous risk, such as those with a personal or familial history of GCA/PMR. Whether the presence of the DRB1*13:01 allele further increases the risk of post-IV GCA/PMR through a stronger vaccine-induced immune reaction deserves further investigation. Unlike PMR, GCA can be a serious complication of IV., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Large vessel involvement in giant cell arteritis has long been described, although its right frequency and potential prognostic value have only been highlighted for two decades. Large vessel involvement not only is associated with a high incidence of late aortic aneurysms, but also might cause greater resistance to glucocorticoids and longer treatment duration, as well as worse late cardiovascular outcomes. These data were brought to our attention, thanks to substantial progress recently made in large vessel imaging. This relies on four single, often complementary, approaches of varying availability: colour Doppler ultrasound, contrast-enhanced computed tomography with angiography and, magnetic resonance imaging, which all demonstrate homogeneous circumferential wall thickening and describe structural changes; 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET/CT), which depicts wall inflammation and assesses many vascular territories in the same examination. In addition, integrated head-and-neck PET/CT can accurately and reliably diagnose cranial arteritis. All four procedures exhibit high diagnostic performance for a large vessel arteritis diagnosis so that the choice is left to the physician, depending on local practices and accessibility; the most important is to carry out the chosen modality without delay to avoid false or equivocal results, due to early vascular oedema changes as a result of high dose glucocorticoid treatment. Yet, ultrasound study of the superficial cranial and subclavian/axillary arteries remains a first line assessment aimed at strengthening and expediting the clinical diagnosis as well as raising suspicion of large-vessel involvement. In treated patients, vascular imaging results are poorly correlated with clinical-biological controlled disease so that it is strongly recommended not to renew imaging studies unless a large vessel relapse or complication is suspected. On the other hand, a structural monitoring of aorta following giant cell arteritis is mandatory, but uncertainties remain regarding the best procedural approach, timing of first control and spacing between controls. Individuals at greater risk of developing aortic complication, e.g. those with classic risk factors for aneurysm and/or visualised aortitis, should be monitored more closely., (Copyright © 2020. Published by Elsevier Masson SAS.)

Objective: Giant cell arteritis (GCA) is the most common primary vasculitis affecting the elderly population. GCA preferentially involves the extracranial branches of the carotid artery; intracranial vasculitis is thought to be a rare occurrence. This study determined the prevalence of intracranial vasculitis in a large series of patients evaluated for GCA and describes the clinical presentation of such cases., Design: Retrospective chart review using a prospective database. When possible, subjects underwent high-resolution 3T contrast-enhanced magnetic resonance imaging (MRI) and MR angiography (MRA) of the scalp and intracranial arteries., Participants: Patients presenting with suspected GCA between January 2015 and December 2018. Four additional, non-database cases of GCA with intracranial involvement are also described., Results: Of 197 patients, 168 had a contrast-enhanced MRI of the head and 51 had imaging findings suggestive of vasculitis. Five patients showed probable or definitive involvement of both the anterior and posterior intracranial circulation with isolated posterior intracranial circulation involvement in one additional patient. One of these patients showed evidence of acute posterior circulation ischemia and presented with vertigo but no evidence of ischemic optic neuropathy or ophthalmic artery enhancement. Of the 51 patients, 14 had abnormal enhancement of the ophthalmic arteries, including 1 with arteritic ischemic anterior optic neuropathy and vertebral arteritis and 1 patient with involvement of the internal carotid and posterior cerebral arteries but no reported vision changes., Conclusion: Although uncommon, clinicians should be aware that GCA can directly involve the intracranial circulation with both the anterior and posterior circulation affected in most of our cases., (Copyright © 2020 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Giant cell arteritis is the most common primary vasculitis of large-vessel occurring in subjects over 50 years of age. Many imaging techniques has been evaluated to improve the diagnosis of giant cell arteritis. Among these imaging techniques, ultrasound has shown good performances to detect inflammatory involvement of the temporal arteries as well as branches of the aorta. Several publications and recent EULAR recommendations have emhasized the place of this tool in the diagnosis of giant cell arteritis., (Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Objective: Giant cell arteritis (GCA) is a systemic vasculitis, affecting medium- and large-sized vessels. Temporal artery biopsies (TABs) are currently the benchmark for diagnosing suspected cases of GCA. Often, the temporal artery is "skeletonized," and surrounding soft tissue is discarded at the time of biopsy. The purpose of this study was to identify cases in which diagnoses were made through examination of periadventitial soft tissue in nonskeletonized TABs., Design: Retrospective observational case series and literature review., Participants: Six patients were recruited on a case-by-case basis., Inclusion Criteria: bilateral TABs for suspected GCA and periadventitial findings leading to appropriate diagnosis., Exclusion Criteria: none., Methods: A retrospective chart review was performed to collect patient demographics and clinical information in 2 academic institutions in Ontario, Canada. The primary outcome measure was identification of histopathological diagnoses made through examination of periadventitial soft tissue in TABs performed for suspected GCA., Results: Two patients were diagnosed with GCA, one of which had a concurrent diagnosis of chronic lymphocytic leukemia. Four patients were diagnosed with small vessel vasculitis, 3 of which were antineutrophil cytoplasmic antibody-related vasculitides. All patients had evidence of a disease limited to the periadventitial tissue of the TAB. The results are limited by the study's size., Conclusions: This case series demonstrates that other serious conditions may declare themselves with symptoms similar to that of GCA. We recommend that surgeons perform a nonskeletonized TAB in all cases of suspected GCA and that pathologists adequately examine the periadventitial tissue in these biopsies to ensure appropriate diagnosis., (Copyright © 2019 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Introduction: Weight loss, myalgias, neurologic manifestations and arterial hypertension are common features of polyarteritis nodosa (PAN) at diagnosis. Temporal arteritis is a rarer manifestation of PAN, more suggestive of giant cell arteritis (GCA)., Case: We report the case of a 77-year-old woman who presented with fatigue, weight loss, fever, neck pain, jaw claudication and cough, diagnosed with giant cell arteritis. Diagnosis was reconsidered in favour of a medium and small-sized vessels necrotizing vasculitis corresponding to PAN because of steroid dependence, mononeuritis and suggestive histological features., Conclusion: Although temporal arteritis is suggestive of GCA, other causes of temporal arteritis can be identified with temporal artery biopsy., (Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Background: Age at onset of large-vessel vasculitis (LVV) is commonly used to distinguish giant cell arteritis (GCA) and Takayasu arteritis (TA). However, LVV between age 50 and 60 years may be difficult to classify., Methods: We conducted a retrospective study including LVV aged between 50 and 60 years at onset (LVV 50-60 , cases) and compared them to LVV aged over 60 years (LVV >60 , controls). LVV was defined histologically and/or morphologically. Controls fulfilled ACR 1990 criteria for GCA or presented isolated aortitis., Results: We included 183 LVV 50-60 and 183 gender-matched LVV >60 . LVV 50-60 had more frequent peripheral limb manifestations (23 vs. 5%), and less frequent cephalic (73 vs. 90%) and ocular signs (17 vs. 27%) than LVV >60 . Compared to LVV >60 , CT angiography and PET/CT scan were more frequently abnormal in LVV 50-60 (74 vs. 38%, and 90 vs. 72%, respectively), with aorta being more frequently involved (78 vs. 47%). By multivariate analysis, absence of cephalic symptoms, presence of peripheral limb ischemia and aorta involvement, and increased CRP level were significantly associated with LVV 50-60 presentation compared to LVV >60 . At last follow-up, compared to LVV >60 , LVV 50-60 received significantly more lines of treatment (2 vs. 1), more frequent biologics (12 vs. 3%), had more surgery (10 vs. 0%), and had higher prednisone dose (8.8 vs. 6.5 mg/d) at last follow-up, CONCLUSION: LVV onset between 50 and 60 years identifies a subset of patients with more frequent aorta and peripheral vascular involvement and more refractory disease compared to patients with LVV onset after 60., (Copyright © 2019. Published by Elsevier B.V.)