Positive Temporal Artery Biopsy Research Articles

Vasculitis associated with VEXAS syndrome.

To define the prevalence, distribution, and characteristics of patients with VEXAS who have confirmed vasculitis. Patients with VEXAS syndrome, verified by positive UBA1 mutation, were included. Chart review was performed to identify. characteristics and outcomes. Vasculitis diagnosis was based on either histopathology showing vascular inflammation or non-invasive angiography findings. Summary statistics were calculated. Eighty-nine patients met inclusion criteria. All were male with a median age of onset of 66.9 years (IQR 60.1, 72.7). Median (IQR) follow up was 3.8(2.2-5.5) years during which 21 patients (23.6%) had evidence of vasculitis. Vasculitis subtypes included small vessel vasculitis (19.1%), cutaneous medium vessel vasculitis (2.2%), and large vessel vasculitis (2.2%). No patient had more than one vessel size involved. Histopathology in small vessel vasculitis patients was consistent with cutaneous leukocytoclastic vasculitis in the majority, though one patient had leukocytoclastic peritubular capillaritis on renal biopsy. Cranial symptoms (headache, vision changes, or jaw pain) were noted in 18.0%. Two additional patients not experiencing cranial symptoms exhibited large vessel involvement with confirmed carotid thickening on non-invasive angiography; one of these had a positive temporal artery biopsy. VEXAS syndrome manifests as a variable vessel vasculitis in a quarter of patients, with cutaneous small and medium vessel involvement being particularly common. Some patients may have positive ANCA serologies or even renal vasculitis leading to misdiagnosis. Cranial symptoms are common and may mimic giant cell arteritis, though documented large vessel inflammation is rare.

Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study

Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older. Current options for diagnosis and treatment are scarce, highlighting the need to better understand its underlying pathogenesis. Genome-wide association studies (GWAS) have emerged as a powerful tool for unravelling the pathogenic mechanisms involved in complex diseases. We aimed to characterise the genetic basis of giant cell arteritis by performing the largest GWAS of this vasculitis to date and to assess the functional consequences and clinical implications of identified risk loci. We collected and meta-analysed genomic data from patients with giant cell arteritis and healthy controls of European ancestry from ten cohorts across Europe and North America. Eligible patients required confirmation of giant cell arteritis diagnosis by positive temporal artery biopsy, positive temporal artery doppler ultrasonography, or imaging techniques confirming large-vessel vasculitis. We assessed the functional consequences of loci associated with giant cell arteritis using cell enrichment analysis, fine-mapping, and causal gene prioritisation. We also performed a drug repurposing analysis and developed a polygenic risk score to explore the clinical implications of our findings. We included a total of 3498 patients with giant cell arteritis and 15 550 controls. We identified three novel loci associated with risk of giant cell arteritis. Two loci, MFGE8 (rs8029053; p=4·96 × 10-8; OR 1·19 [95% CI 1·12-1·26]) and VTN (rs704; p=2·75 × 10-9; OR 0·84 [0·79-0·89]), were related to angiogenesis pathways and the third locus, CCDC25 (rs11782624; p=1·28 × 10-8; OR 1·18 [1·12-1·25]), was related to neutrophil extracellular traps (NETs). We also found an association between this vasculitis and HLA region and PLG. Variants associated with giant cell arteritis seemed to fulfil a specific regulatory role in crucial immune cell types. Furthermore, we identified several drugs that could represent promising candidates for treatment of this disease. The polygenic risk score model was able to identify individuals at increased risk of developing giant cell arteritis (90th percentile OR 2·87 [95% CI 2·15-3·82]; p=1·73 × 10-13). We have found several additional loci associated with giant cell arteritis, highlighting the crucial role of angiogenesis in disease susceptibility. Our study represents a step forward in the translation of genomic findings to clinical practice in giant cell arteritis, proposing new treatments and a method to measure genetic predisposition to this vasculitis. Institute of Health Carlos III, Spanish Ministry of Science and Innovation, UK Medical Research Council, and National Institute for Health and Care Research.

Upper respiratory tract and orofacial manifestations of new-onset giant cell arteritis: results from a large, prospective inception cohort study.

Giant cell arteritis (GCA) often features upper respiratory tract (URT) and orofacial manifestations, which signal the involvement of external carotid artery branches. In this study, we aimed to describe the frequency of various URT/orofacial symptoms at GCA onset, as well as the main characteristics of patients presenting these symptoms. We included all patients who were newly diagnosed with GCA between 1976 and April 2022 at the Internal Medicine Department of a tertiary-care hospital. Ten URT or orofacial symptoms were prospectively examined systematically in each patient. We used multivariate analyses to identify the GCA characteristics, including URT/orofacial symptoms, associated with temporal artery biopsy (TAB) positivity. At least 1 URT/orofacial symptom was present in 68.6% of the 599 patients (3 or more symptoms in 30% of cases). Jaw claudication, maxillary pain, and pain during mouth opening were the most prevalent symptoms. Dry cough was recorded in 17% of cases. GCA patients with URT/orofacial symptoms had more clinical abnormalities of the temporal artery bed and ischaemic ophthalmological complications, but less large-vessel vasculitis according to imaging. The likelihood of a positive TAB was increased in patients with an abnormal temporal artery upon clinical examination (OR 4.16; CI 2.75-6.37, p < 0.001) or jaw claudication (OR 2.18; CI 1.35-3.65, p = 0.002), and decreased in those with hoarseness (OR 0.47; CI 0.26-0.87, p = 0.02) or earache (OR 0.54; CI 0.31-0.95, p = 0.03). Isolated URT/orofacial presentation (i.e., without headache or visual signs) accounted for 5.2% of the entire cohort. Oral-facial symptoms were present in two-thirds of GCS cases. Thus, they could serve as leading clinical clues for a GCA diagnosis, and are a risk factor for permanent visual loss. Several URT/orofacial symptoms such as jaw claudication, hoarseness, and earache influenced the likelihood of a positive TAB. Isolated URT/orofacial presentation of GCA is a rare but potentially challenging occurrence.

Giant cell arteritis: incidence and phenotypic distribution in Western Norway 2013-2020.

There is an increasing awareness of the spectrum of phenotypes in giant cell arteritis (GCA). However, there is sparse evidence concerning the phenotypic distribution which may be influenced by both genetic background and the environment. We established a cohort of all GCA-patients in the Bergen Health Area (Western Norway), to describe the phenotypic distribution and whether phenotypes differ with regards to incidence and clinical features. This is a retrospective cohort study including all GCA-patients in the Bergen Health Area from 2013-2020. Data were collected by reviewing patient records, and patients considered clinically likely GCA were included if they fulfilled at least one set of classification criteria. Temporal artery biopsy (TAB) and imaging results were used to classify the patients according to phenotype. The phenotype "cranial GCA" was used for patients with a positive TAB or halo sign on temporal artery ultrasound. "Non-cranial GCA" was used for patients with positive findings on FDG-PET/CT, MRI-, or CT angiography, or wall thickening indicative of vasculitis on ultrasound of axillary arteries. Patients with features of both these phenotypes were labeled "mixed." Patients that could not be classified due to negative or absent examination results were labeled "unclassifiable". 257 patients were included. The overall incidence of GCA was 20.7 per 100,000 persons aged 50 years or older. Overall, the cranial phenotype was dominant, although more than half of the patients under 60 years of age had the non-cranial phenotype. The diagnostic delay was twice as long for patients of non-cranial and mixed phenotype compared to those of cranial phenotype. Headache was the most common clinical feature (78% of patients). Characteristic clinic features occurred less frequently in patients of non-cranial phenotype compared to cranial phenotype. The overall incidence for GCA was comparable to earlier reports from this region. The cranial phenotype dominated although the non-cranial phenotype was more common in patients under 60 years of age. The diagnostic delay was longer in patients with the non-cranial versus cranial phenotype, indicating a need for examination of non-cranial arteries when suspecting GCA.

Giant cell temporal arteritis: a clinicopathological study with emphasis on unnecessary biopsy.

Temporal artery (TA) biopsy is commonly used for the diagnosis of giant cell arteritis (GCA). However, a positive biopsy is no longer mandatory for diagnosis. This study aims to correlate the histopathological findings of TA biopsies in suspected cases of GCA to the clinical presentation in an ophthalmic tertiary eye care center to draw useful conclusions and advocate the possible implementation of guidelines for TA biopsy. Data was collected from patients' medical records including, demographics, clinical data, and histopathological findings and diagnosis. The 2022 American College of Rheumatology/ European Alliance of Associations for Rheumatology (ACR/EULAR) criteria have been used and partially adopted as a guide to compare the variables between TA biopsy-positive and negative groups as well as the TA biopsy-positive group and the group of patients with TA biopsy showing atherosclerosis. Out of the total 35 patients who underwent a TA biopsy during the period of 23 years, 22.9% of patients had histopathological findings consistent with GCA and 42.9% had TA atherosclerotic changes, while the remaining 34.3% had histologically unremarkable TA. The mean age of all patients was 66 ± 10.9 years. Slightly more than half were females (54.3%) and the remaining were males (45.7%). In the group with positive TA biopsies, the mean age was 71 ± 8.4 years with a higher female predominance (female-to-male ratio of 5:3). The mean diagnostic clinical score used in our study was higher (7.5 ± 2.33) in the GCA-positive group when compared to the other groups with statistical significance (mean of 4.85 ± 2.01 in patients with overall GCA-negative biopsies and 5.13 ± 2.10 in the group with atherosclerosis). Other three clinical variables that were found to be statistically significant in the GCA biopsy-positive group were scalp tenderness, jaw claudication, and optic nerve pallor. The mean age (71 ± 8.4 years) and the female predominance of GCA in our group of patients with positive TA biopsy (62.5%) was like other reports. In our study 22.9% of performed TA biopsies over the period of the study were positive confirming the diagnosis of GCA on histological exam, which was similar to another report and is considered to be relatively low. The incorporation of increased clinically focused assessments and algorithms, with the aid of the ACR/EULAR criteria, may decrease the frequency of TA biopsies that carries unnecessary cost and risk of procedure-related morbidity. We highly recommend applying the age of ≥ 50 years as an initial criterion for diagnosis, followed by the consideration of the statistically significant clinical features: scalp tenderness, jaw claudication, and optic nerve pallor.

Estimating overdiagnosis in giant cell arteritis diagnostic pathways using genetic data: genetic association study.

GCA can be confirmed by temporal artery biopsy (TAB) but false negatives can occur. GCA may be overdiagnosed in TAB-negative cases, or if neither TAB nor imaging is done. We used HLA genetic association of TAB-positive GCA as an 'unbiased umpire' test to estimate historic overdiagnosis of GCA. Patients diagnosed with GCA between 1990 and 2014 were genotyped. During this era, vascular imaging alone was rarely used to diagnose GCA. HLA region variants were jointly imputed from genome-wide genotypic data of cases and controls. Per-allele frequencies across all HLA variants with P < 1.0 × 10-5 were compared with population control data to estimate overdiagnosis rates in cases without a positive TAB. Genetic data from 663 GCA patients were compared with data from 2619 population controls. TAB-negative GCA (n = 147) and GCA without TAB result (n = 160) had variant frequencies intermediate between TAB-positive GCA (n = 356) and population controls. For example, the allele frequency of HLA-DRB1*04 was 32% for TAB-positive GCA, 29% for GCA without TAB result, 27% for TAB-negative GCA and 20% in population controls. Making several strong assumptions, we estimated that around two-thirds of TAB-negative cases and one-third of cases without TAB result may have been overdiagnosed. From these data, TAB sensitivity is estimated as 88%. Conservatively assuming 95% specificity, TAB has a negative likelihood ratio of around 0.12. Our method for utilizing standard genotyping data as an 'unbiased umpire' might be used as a way of comparing the accuracy of different diagnostic pathways.

Giant cells in temporal artery biopsies and the risk of large vessel involvement in patients with giant cell arteritis.

Giant cell arteritis (GCA) is a systemic disease with variable vascular involvement. The objective was to investigate predictors of time-dependent large vessel involvement (LVI) in a population-based cohort of patients with GCA. GCA patients with positive temporal artery biopsies (TAB) between 1997- 2010 were identified through a regional pathology register. A structured review of histopathology reports and relevant imaging studies was performed. Cases with LVI through July 2016 were identified. Patients were followed to first LVI, death, migration from the area or July 29, 2016. Event free survival by clinical and histopathologic features was estimated using the Kaplan-Meier method. Potential predictors of LVI were examined using Cox regression models. A total of 274 patients were included. The mean age at GCA diagnosis was 75.7 years. Fifty-one patients (19 %) had documented LVI during the follow-up, corresponding to an incidence rate of 2.4/100 person-years. The median time from GCA diagnosis to the diagnosis of LVI was 4.5 years (interquartile range 0.6-7.4). Thirty-four patients had aortic involvement (67% of those with LVI; 12% of all GCA cases). Survival free of LVI was longer in patients with giant cells in the TAB (75th percentile 14.0 vs 6.7 years; p=0.014). In age-adjusted analysis, the presence of giant cells in the TAB was associated with reduced risk of LVI (hazard ratio 0.48; 95 % confidence interval 0.27-0.86). The negative association with giant cells in the TAB suggests that patients with LVI constitute a subset of GCA with particular disease mechanisms.

Assessment and comparison of probability scores to predict giant cell arteritis

Introduction/objectivesTo assess and compare the performance of the giant cell arteritis probability score (GCAPS), Ing score, Bhavsar-Khalidi score (BK score), color Doppler ultrasound (CDUS) halo count, and halo score, to predict a final diagnosis of giant cell arteritis (GCA).MethodA prospective cohort study was conducted from April to December 2021. Patients with suspected new-onset GCA referred to our quaternary CDUS clinic were included. Data required to calculate each clinical and CDUS probability score was systematically collected at the initial visit. Final diagnosis of GCA was confirmed clinically 6 months after the initial visit, by two blinded vasculitis specialists. Diagnostic accuracy and receiver operator characteristic (ROC) curves for each clinical and CDUS prediction scores were assessed.ResultsTwo hundred patients with suspected new-onset GCA were included: 58 with confirmed GCA and 142 without GCA. All patients with GCA satisfied the 2022 ACR/EULAR classification criteria. A total of 5/15 patients with GCA had a positive temporal artery biopsy. For clinical probability scores, the GCAPS showed the best sensitivity (Se, 0.983), whereas the BK score showed the best specificity (Sp, 0.711). As for CDUS, a halo count of 1 or more was found to have a Se of 0.966 and a Sp of 0.979. Combining concordant results of clinical and CDUS prediction scores showed excellent performance in predicting a final diagnosis of GCA.ConclusionUsing a combination of clinical score and CDUS halo count provided an accurate GCA prediction method which should be used in the setting of GCA Fast-Track clinics.Key Points• In this prospective cohort of participants with suspected GCA, 3 clinical prediction tools and 2 ultrasound scores were compared head-to-head to predict a final diagnosis of GCA.• For clinical prediction tools, the giant cell arteritis probability score (GCAPS) had the highest sensitivity, whereas the Bhavsar-Khalidi score (BK score) had the highest specificity.• Ultrasound halo count was both sensitive and specific in predicting GCA.• Combination of a clinical prediction tool such as the GCAPS, with ultrasound halo count, provides an accurate method to predict GCA.

Clinical phenotype and complications of large vessel giant cell arteritis: A systematic review and meta-analysis.

Giant Cell Arteritis (GCA) is a heterogenous systemic granulomatous vasculitis involving the aorta and any of its major tributaries. Despite increased awareness of large vessel (LV) involvement, studies reporting incidence, clinical characteristics and complications of large-vessel GCA (LV-GCA) show conflicting results due to inconsistent disease definitions, differences in study methodologies and the broad spectrum of clinical presentations. The aim of this systematic literature review was to better define LV-GCA based on the available literature and identify distinguishing characteristics that may differentiate LV-GCA patients from those with limited cranial disease. Published studies indexed in MEDLINE and EMBASE were searched from database inception to 7th May 2021. Studies were included if they presented cohort or cross-sectional data on a minimum of 25 patients with LV-GCA. Control groups were included if data was available on patients with limited cranial GCA (C-GCA). Data was quantitatively synthesised with application of a random effects meta-regression model, using Stata. The search yielded 3488 studies, of which 46 were included. Diagnostic criteria for LV-GCA differed between papers, but was typically dependent on imaging or histopathology. Patients with LV-GCA were generally younger at diagnosis compared to C-GCA patients (mean age difference -4.53years), had longer delay to diagnosis (mean difference 3.03months) and lower rates of positive temporal artery biopsy (OR: 0.52 [95% CI: 0.3, 0.91]). Fewer LV-GCA patients presented with cranial manifestations and only 53% met the 1990 ACR Classification Criteria for GCA. Vasculitis was detected most commonly in the thoracic aorta, followed by the subclavian, brachiocephalic trunk and axillary arteries. The mean cumulative prednisolone dose at 12-months was 6056.5mg for LV-GCA patients, relapse rates were similar between LV- and C-GCA patients, and 12% of deaths in LV-GCA patients could be directly attributed to an LV complication. Patients with LV-GCA have distinct disease features when compared to C-GCA, and this has implications on diagnosis, treatment strategies and surveillance of long-term sequalae.

Characteristics and Prognosis of Binocular Diplopia in Patients With Giant Cell Arteritis.

Giant cell arteritis (GCA) is a large vessel vasculitis associated with a risk of permanent ophthalmologic complications. Data about diplopia prognosis in GCA are scarce. This study was designed to better characterize diplopia in newly diagnosed GCA patients. All consecutive patients diagnosed with GCA from January 2015 to April 2021 in a French tertiary ophthalmologic center were retrospectively reviewed. GCA diagnosis relied on a positive temporal artery biopsy or high-definition MRI. Among 111 patients diagnosed with GCA, 30 patients (27%) had diplopia. Characteristics of patients with diplopia were similar to other GCA patients. Diplopia resolved spontaneously in 6 patients (20%). Diplopia was attributed to cranial nerve palsy in 21/24 patients (88%), especially third (46%) and sixth cranial nerve (42%). Ocular ischemic lesions occurred in 11 of the 30 patients with diplopia (37%); 2 patients developed vision loss after initiation of corticosteroids. In the remaining 13 patients, diplopia resolved after treatment onset in 12 patients (92%) with a median delay of 10 days. Patients treated intravenously tended to have a quicker improvement than those treated orally, but with a similar resolution rate of diplopia at 1 month. Two patients had relapse of diplopia at 4 and 6 weeks after an initial treatment course of 24 and 18 months, respectively. Diplopia is a rare feature at GCA diagnosis, but should raise clinician suspicion for GCA when associated with cephalic symptoms and prompt the initiation of corticosteroids to prevent ocular ischemic complications.

Clinical Predictors of Pathologically Positive Temporal Artery Biopsy

Temporal arteritis (TA) can present with a wide spectrum of symptoms and lab findings, including temporal headaches, vision changes, jaw or tongue claudication, fevers, and elevated serum inflammatory markers. Here, we review a large series of patients who have undergone temporal artery biopsies. A retrospective review of all patients who underwent temporal artery biopsies within our health system from January 2005 to January 2022 was performed. Patients were selected using the Current Procedural Terminology code for temporal artery biopsy (37609). Chart review was performed to determine demographic, clinical, laboratory, and operative characteristics, as well as pathologic outcome. The primary outcome was the final pathology result of the biopsy. Patients with non-diagnostic pathology reports were excluded from the study. Variables that met a P-value < .1 in univariate analysis were included in the final multivariable logistic regression analysis. A separate sensitivity analysis was performed on a cohort with complete lab data. We identified 613 patients who underwent temporal artery biopsies across three hospitals within our health system from January 2005 to January 2022. The average age of the cohort was 73.2 ± 11.2 years. The majority of the patients, 70.5% (n = 430), were female. The most common presenting symptom was a headache, which was present in 254 (41.4%) of patients. Older age (>65), white race, lower body mass index (BMI), presence of jaw claudication, lack of blurry vision, elevated erythrocyte sedimentation rate, elevated C-reactive protein, elevated platelet count, and longer biopsy length were associated with a positive biopsy on univariate analysis (P < .05) (Table I). Specimen length did not differ significantly between groups with positive and negative pathology, nor by bilateral versus unilateral biopsy or surgeon specialty. On multivariate analysis, the presence of jaw claudication (odds ratio [OR], 3.69; P < .001) was associated with a positive biopsy. Black race was associated with a lower odds of a positive biopsy (OR, 0.27; P < .001). On sensitivity analysis, erythrocyte sedimentation rate levels above 40 were significantly associated with increased odds of a positive biopsy (OR, 7.53; P = .009). Platelet counts of 401 to 600 (OR, 7.60; P < .001) and above 600 (OR, 7.15; P = .006) were also significantly associated with a positive biopsy. Clinical and patient features including the symptom of jaw claudication, elevated erythrocyte sedimentation rate, elevated platelet count, and white race are associated with positive temporal artery biopsy. Further work is needed to determine who benefits most from this procedure.TablePatient characteristics and biopsy outcomesTotalNegative biopsy resultPositive biopsy resultP-valueN = 613n = 523n = 90Age, years <5011 (1.8)10 (1.9)1 (1.1).009 50-64109 (17.8)103 (19.7)6 (6.7) 65-79313 (51.1)266 (50.9)47 (52.2) 80+180 (29.4)144 (27.5)36 (40.0)Sex Female430 (70.5)374 (71.8)56 (62.9).090 Male180 (29.5)147 (28.2)33 (37.1)Race/ethnicity White322 (52.8)250 (48.1)72 (80.0)<.001 Black229 (37.5)216 (41.5)13 (14.4) Other/unknown59 (9.7)54 (10.4)5 (5.6)BMI Underweight (BMI <18.5)11 (1.8)7 (1.3)4 (4.4)<.001 Healthy (18.5 ≤ BMI < 25)168 (27.4)123 (23.5)45 (50.0) Overweight (25 ≤ BMI < 30)172 (28.1)150 (28.7)22 (24.4) Obese (BMI ≥30)262 (42.7)243 (46.5)19 (21.1)Preoperative steroid use Yes392 (68.7)330 (67.8)62 (73.8).27Preoperative aspirin use Yes254 (45.3)218 (45.5)36 (43.9).79Past medical history of other rheumatic disease Yes147 (25.2)130 (26.1)17 (20.2).26History of polymyalgia rheumatica Yes62 (26.6)53 (26.4)9 (28.1).83Bruit Yes3 (0.5)3 (0.6)0 (0.0).47Fever Yes41 (6.7)32 (6.1)9 (10.0).17Headache Yes254 (41.4)216 (41.3)38 (42.2).87Tenderness Yes130 (21.2)111 (21.2)19 (21.1).98Bilateral tenderness Yes26 (4.2)19 (3.6)7 (7.8).072Jaw claudication Yes95 (15.6)64 (12.3)31 (34.8)<.001Tongue claudication Yes0 (0.0)0 (0.0)0 (0.0)Blurry vision Yes111 (18.1)102 (19.5)9 (10.0).030Double vision Yes18 (2.9)13 (2.5)5 (5.6).11Tinnitus Yes6 (1.0)5 (1.0)1 (1.1).89Erythrocyte sedimentation rate at diagnosis <2050 (9.4)48 (10.6)2 (2.5).008 20-4059 (11.1)55 (12.1)4 (5.1) 40+423 (79.5)350 (77.3)73 (92.4)C-reactive protein at diagnosis <3140 (33.5)128 (36.1)12 (19.0)<.001 3-1083 (19.9)68 (19.2)15 (23.8) 11-2060 (14.4)58 (16.3)2 (3.2) 20+135 (32.3)101 (28.5)34 (54.0)Platelet count at diagnosis <200112 (20.5)104 (22.3)8 (9.9)<.001 200-400356 (65.1)314 (67.4)42 (51.9) 401-60063 (11.5)38 (8.2)25 (30.9) 601-800+16 (2.9)10 (2.1)6 (7.4)Alk-Phos (continuous)93.1 (65.9)92.9 (66.8)94.2 (62.1).90White blood cells (continuous)11.0 (17.8)10.8 (19.0)11.6 (8.1).71Longest length of biopsy specimen, cm (continuous)1.9 (0.9)1.9 (0.9)2.1 (1.0).057Boldface P indicates P-value < .1, used in multivariate analysis.Data are presented as number (%) or mean (standard deviation). Open table in a new tab

OA34 To determine the diagnostic test accuracy of different ultrasound diagnostic criteria in the diagnosis of giant cell arteritis: a secondary data analysis

Abstract Background/Aims To determine the diagnostic test accuracy of compression ultrasound compared to combined compression and halo techniques in the diagnosis of giant cell arteritis. Methods A retrospective secondary data analysis was conducted of 111 consecutive patients referred with suspected giant cell arteritis (GCA). All patients had an ultrasound of superficial temporal arteries using halo and compression techniques. All had a temporal artery biopsy (TAB), directed by clinical suspicion. Diagnosis was confirmed at six months based on clinical diagnosis. Exclusion criteria included flare, or relapsing patients. Two by two frequency tables were used to calculate sensitivity, specificity, positive and negative predictive values, positive and negative likelihood ratios of compression and combined halo and compression in comparison to TAB results and clinical diagnosis at six months. Multiple logistic regressions were calculated adjusting for the identified confounders of gender, steroid dose at time of ultrasound and ischemic symptoms of jaw claudication, transient or permanent vision loss. Local NHS Trust Caldicott approval was given for this study. Results Of the 111 patients referred, 56 were clinically diagnosed with GCA. A positive TAB result was recorded in 33. Compression ultrasound had a high sensitivity of 90.6 % (95%CI 75.0, 98.0) but a low specificity of 32.9 % (95% CI 22.8, 44.4) when compared to TAB. Compression had a sensitivity of 83.6% (95% CI 71.2, 92.2) and specificity 34.5% (95% CI 22.2, 48.6) when compared to clinical diagnosis. This is in contrast to compression and halo combined, which had a sensitivity of 71.9% (95% CI 53.3, 86.3) when compared to TAB and 56.4 % (95% CI 42.3.69.7) when compared to clinical diagnosis. However, specificity of the combined techniques was much higher (73.4% [95% CI 62.3, 82.7] and 76.4% [95% CI 62.3, 86.38]) when compared to TAB and clinical diagnosis respectively. A summary of results can be seen in Table 1. Conclusion Compression ultrasound has a high sensitivity; this coupled with the relatively high negative predictive value suggests compression ultrasound may be useful in screening and stratification of patient management. The perceived ease of compression ultrasound suggests further evaluation of compression ultrasound in clinical practice is required. Disclosure K. Smith: Grants/research support; HEE/NIHR Pre-doctoral clinical academic fellowship. B. Sarker: None. J. Lim: None. C. Burnett: None. D. Taze: None. A. Chakrabarty: None. R.J. Wakefield: None. S.L. Mackie: Consultancies; SLM has provided consultancy on behalf of her institution for Roche/Chugai, Sanofi, AbbVie and AstraZeneca. Honoraria; SLM has given talks on behalf of her institution for Pfizer, Vifor and UCB. In all cases fees were paid to her institution and no personal fees were received. Grants/research support; SLM has been an investigator on clinical trials for Sanofi and GSK. As a research grant co-applicant, SLM has received research funding (partial salary support) from Vifor, paid to her institution.

The Effect of Corticosteroids on Temporal Artery Biopsy Positivity in Giant Cell Arteritis: Timing is Everything.

Temporal artery biopsy (TAB) remains the standard criterion for the diagnosis of giant cell arteritis (GCA). Temporal artery biopsy is suggested to be performed within 2 weeks from the initiation of corticosteroids. However, the effects of TAB timing on the sensitivity of its findings still warrant further investigation. We reviewed the medical records of patients with GCA from a tertiary medical center in Germany over an 8-year period. We analyzed data from 109 patients with a median age of 76 years and a median time from glucocorticoid treatment to TAB of 4 days. Approximately 60% of biopsies were positive. Our analysis yielded a nonsignificant trend toward shorter duration of corticosteroid treatment in the TAB(+) group ( p = 0.06). A more than 7 days' duration of steroid treatment was independently linked with lower rates of positive TAB (adjusted odds ratio, 0.33; 95% confidence interval, 0.11-1.00). We conclude that the duration of corticosteroid treatment seems to affect the positivity of TAB in patients with suspected GCA. Further larger studies are required to confirm the generalizability of our findings.

Validating the Clinical Value of Temporal Artery Biopsy

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. A retrospective review at a single-center tertiary care hospital was performed between 2007 and 2018. The inclusion criteria were patients greater than 18years 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. 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 30days, 49% (37) after 90days and 45% (34) after 180days. 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 787days respectively; P=0.682). Average follow up was 33months. 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.

Influence of the EULAR recommendations for the use of imaging in large vessel vasculitis in the diagnosis of giant cell arteritis: results of the ARTESER register

ObjectiveThe main study objective was to determine how giant cell arteritis (GCA) is diagnosed in our clinical practice and whether the EULAR recommendations have influenced the diagnostic procedures used.MethodsARTEritis of...

Temporal Artery Biopsy Debate: Positive TAB Result Prolongs Steroid Use in Giant Cell Arteritis.

A retrospective chart review garnered patient demographics, symptoms, comorbidities, and steroid treatment duration in patients undergoing TAB at a single center. Steroid treatment was compared between TAB+ and TAB - patients. One hundred seven patients undergoing TAB were included. Patients were predominantly women (70.1%) with a median age of 74 years (46 -91). Of 107 TAB results, 74 (69.2%) were negative, 23 (21.5%) were positive, and 10 (9.3%) were found to be indeterminate. In TAB+ patients, the mean erythrocyte sedimentation rate was not significantly different than TAB - patients (60.2 versus 43.7, P = 0.45), nor was the median C-reactive protein (38.8 versus 18.1, P = 0.17). Regarding steroid use, both TAB+ and TAB - patients had a similarly high rate of prebiopsy steroid initiation (82.6% versus 70.3%, P = 0.32). More TAB+ patients remained on steroids at 6 weeks (95.0% versus 57.4%, P = 0.004), 6 months (95% versus 37.7%, P < 0.001), 1 year (65.0% versus 31.1%, P = 0.024), and 18 months (50.0% versus 19.7%, P = 0.045). By 2 years, the difference no longer met significance (35.0% versus 14.8%, P = 0.12). P = 0.12). TAB positivity does seem to influence maintenance of steroids up to 18 months after biopsy.

2022 American College of Rheumatology/EULAR Classification Criteria for Giant Cell Arteritis.

To develop and validate updated classification criteria for giant cell arteritis (GCA). Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate items, 2) prospective collection of candidate items present at the time of diagnosis, 3) expert panel review of cases, 4) data-driven reduction of candidate items, 5) derivation of a points-based risk classification score in a development data set, and 6) validation in an independent data set. The development data set consisted of 518 cases of GCA and 536 comparators. The validation data set consisted of 238 cases of GCA and 213 comparators. Age ≥50 years at diagnosis was an absolute requirement for classification. The final criteria items and weights were as follows: positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5); erythrocyte sedimentation rate ≥50 mm/hour or C-reactive protein ≥10 mg/liter (+3); sudden visual loss (+3); morning stiffness in shoulders or neck, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality on vascular examination, bilateral axillary involvement on imaging, and fluorodeoxyglucose-positron emission tomography activity throughout the aorta (+2 each). A patient could be classified as having GCA with a cumulative score of ≥6 points. When these criteria were tested in the validation data set, the model area under the curve was 0.91 (95% confidence interval [95% CI] 0.88-0.94) with a sensitivity of 87.0% (95% CI 82.0-91.0%) and specificity of 94.8% (95% CI 91.0-97.4%). The 2022 American College of Rheumatology/EULAR GCA classification criteria are now validated for use in clinical research.

A nationwide study of ocular manifestations leading to hospital contacts among patients with giant cell arteritis

ObjectivesTo investigate the risk of ocular manifestations leading to hospital contacts among patients with giant cell arteritis (GCA). MethodsA Danish, nationwide, register-based cohort study including 14,574 GCA patients diagnosed 1996–2018 and 145,740 general population referents, matched on sex and date of birth. Cumulative incidence proportions (CIPs) and relative risks (RRs) of ocular manifestations with 95% confidence intervals (CIs) were calculated using a pseudo-observation approach. ResultsA total of 1026/14,574 (7.0%) GCA patients were registered with ocular manifestations within ±1 year of the diagnosis; 392/1026 (38%) being before and 634/1026 (62%) after the GCA diagnosis, and 744/1026 (73%) were registered within ±1 month of the diagnosis. The diagnoses were 336/1026 (33%) retinal vascular occlusions, 300/1026 (29%) disorders of the optic nerve, 177/1026 (17%) visual impairment, 90/1026 (9%) diplopia, and 123/1026 (12%) amaurosis fugax. The CIP for ocular manifestations among GCA patients after 3, 6, and 12 months following the diagnosis were 4.0% (95% CI: 3.6–4.3), 4.2% (95% CI: 3.9–4.6), and 4.6% (95% CI: 4.2–4.9). The 1-year RR of ocular manifestations among GCA patients was 28.0 (95% CI: 24.0–32.7), with age above 70 years, male sex, and a positive temporal artery biopsy being risk factors. Treatment with low-dose aspirin was not associated with a reduced 1-year RR of incident ocular manifestations. ConclusionsIn GCA, most cases of ocular manifestations leading to hospital contacts occur close to the time of diagnosis, with over one-third of cases occurring before the diagnosis, emphasizing the need for early recognition and treatment.

Pericardial effusion in giant cell arteritis is associated with increased inflammatory markers: a retrospective cohort study.

Giant cell arteritis (GCA) is the most frequent vasculitis affecting adults aged > 50years. Cardiac involvement in GCA is considered rare, and only a few cases of pericarditis have been reported. The aim of this study was to determine the characteristics and prognosis of GCA patients suffering from pericardial involvement at diagnosis. We conducted a single-centre, retrospective chart review of patients with GCA in internal medicine departments (from 2000 to 2020). Patients were identified through a centralized hospital database. We retrospectively collected demographic, clinicobiological, histological, imaging, treatment and outcome data. Patients with pericardial effusion, defined as an effusion visible on the CT-scan performed at GCA diagnosis were compared to those without pericardial involvement. Among the 250 patients with GCA, 23 patients (9.2%) had pericardial effusion on CT-scan. The comparison between the groups revealed similar distribution of age, gender, cranial symptoms and ocular ischaemic complications. Patients with pericardial effusion had a higher frequency of weight loss. They also had lower haemoglobin levels and higher platelet levels (p = 0.006 and p = 0.002, respectively), and they more frequently had positive temporal artery biopsy. There were no differences concerning the treatment, relapses, follow-up duration or deaths. This case series sheds light on GCA as a cause of unexplained pericardial effusion or symptomatic pericarditis among adults aged > 50years and elevated inflammatory biological markers. Fortunately, pericardial involvement is a benign GCA manifestation. In that context, the search for constitutional symptoms, cranial symptoms and associated signs of polymyalgia rheumatica is crucial for rapidly guiding GCA diagnosis.

Effect of Temporal Artery Biopsy Length and Laterality on Diagnostic Yield.

Giant cell arteritis (GCA) is the most common vasculitis in adults and is associated with significant morbidity and mortality. Temporal artery biopsy (TAB) remains the gold standard for diagnosis in the United States; however, practices vary in the length of artery obtained and whether bilateral simultaneous biopsies are obtained. Retrospective chart review of all TABs performed at the Johns Hopkins Wilmer Eye Institute between July 1, 2007, and September 30, 2017. Five hundred eighty-six patients underwent TAB to evaluate for GCA. Of 404 unilateral biopsies, 68 (16.8%) were positive. Of 182 patients with bilateral biopsies, 25 (13.7%) had biopsies that were positive and 5 patients (2.7%) had biopsies that were discordant, meaning only 1 side was positive. There was no significant difference in the average postfixation length of positive and negative TAB specimens (positive mean length 1.38 ± 0.61 cm, negative mean length 1.39 ± 0.62 cm, P = 0.9). There is no significant association between greater length of biopsy and a positive TAB result in our data. Although the rate of positive results was not higher in the bilateral group compared with the unilateral group, 2.7% of bilateral biopsies were discordant, similar to previously published rates. Overall, this suggests that initial bilateral biopsy may increase diagnostic yield, albeit by a small amount.