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

Objectives: To investigate the value of [18F]fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) in predicting relapse after treatment discontinuation in patients with large-vessel giant cell arteritis (LV-GCA). Methods: This study included patients with LV-GCA whose treatment was discontinued between 2018 and 2023. All patients underwent PET/CT and/or MRI at the time of treatment discontinuation in clinical remission. Qualitative and quantitative PET/CT scores, by measuring standardized uptake values (SUV), and semiquantitative MRI scores of the aorta and supraaortic vessels were compared between patients who relapsed within 4 months after treatment discontinuation and those who did not. Results: Forty patients were included (median age 67.4 years, interquartile range (IQR) 60.8-74.0; 77.5 % females). Eleven patients (27.5 %) relapsed after treatment discontinuation (time to relapse 1.9 months, IQR 1.4-3.3). Patients who relapsed were comparable to those who remained in remission with respect to the presence of active vasculitis on MRI and/or PET/CT (54.5% vs. 58.6 %, p = 1.0), the number of segments with vasculitic findings on MRI (0, IQR 0.0-1.5, vs. 2, IQR 0.0-3.0, p = 0.221) or the highest SUV artery/liver ratio on PET/CT (1.5, IQR 1.4-1.6, vs. 1.3, IQR 1.2-1.6, p = 0.505). The median number of vasculitic segments on PET/ CT was 2.5 (IQR 0.5-4.5) in those with vs. 0 (IQR 0.0-1.5, p = 0.085) in those without relapse, and the PET/CT scores 4.5 (IQR 0.75-8.25) vs. 0 (IQR 0.0-3.0, p = 0.172). Conclusion: PET/CT or MRI at treatment stop did not predict relapse and may not be suited to guide treatment decisions in patients with LV-GCA in remission.

To the Editor: We read with great interest the new 2022 classification criteria for giant cell arteritis (GCA) proposed by the American College of Rheumatology (ACR) and EULAR (1). As Dr. Ponte et al noted, these updated 2022 classification criteria allow for the correct classification of more patients with the GCA phenotype of extracranial large-vessel vasculitis (LVV) than the previous 1990 ACR classification criteria (2). However, important gaps still exist for the diagnosis of GCA in some patients with predominant extracranial LVV. Indeed, temporal artery biopsy and the temporal artery “halo” sign on ultrasonography are often negative in this phenotype (3). It is really intriguing why such a different clinical presentation may be seen in GCA. It doesn’t seem to be related to genetic factors, since the strong association with the HLA region shown by patients with the classic cranial GCA phenotype has recently been confirmed in patients with extracranial largevessel GCA without cranial ischemic manifestations (4). In the new ACR/EULAR classification criteria for GCA, we see that some patients with a “pure” extracranial LVV phenotype may not be classified as having GCA. This is the case for patients presenting with fever of unknown origin or even more commonly in those with refractory or atypical polymyalgia rheumatica as previously reported (5). Using the new classification criteria, fluorodeoxyglucose–positron emission tomography (FDG-PET) activity in the entire aorta receives 2 points, and the score required to classify a patient with GCA is at least 6 points. Furthermore, regression analysis shown in the supplementary materials of the article by Ponte et al (available at http://onlinelibrary.wiley.com/ doi/10.1002/art.42325) suggests that FDG-PET could be a stronger predictor variable for large-vessel GCA than angiography or ultrasonography (1). Therefore, consideration should be given whether to assign more weight to FDG-PET activity to confirm a diagnosis of GCA in patients presenting with extracranial LVV. Although the advent of new imaging techniques has proven to be useful for identifying patients with large-vessel GCA without the classic cranial manifestations of the disease, there is no uniform consensus on the imaging techniques to be used, which depends on the experience and availability of each center. For this reason, we believe that steps should be taken to reach an agreement on which imaging technique should be used and when, if this phenotype is suspected. Doing so may help to increase the relevance of FDG-PET or other imaging techniques for the diagnosis of GCA. Author disclosures are available at https://onlinelibrary.wiley.com/ action/downloadSupplement?doi=10.1002%2Fart.42439&file=art42439sup-0001-Disclosureform.pdf.

Objectives We evaluated the feasibility of a rapid glucocorticoid tapering regimen to reduce glucocorticoid exposure in patients with giant cell arteritis (GCA) treated with glucocorticoids only. Methods Newly diagnosed patients with GCA treated with a planned 26-week glucocorticoid tapering regimen at the University Hospital Basel were included. Data on relapses, cumulative steroid doses (CSD) and therapy-related adverse effects were collected from patients’ records. Results Of 47 patients (64% women, median age 72 years), 32 patients (68%) had relapsed. Most relapses were minor (28/32) and 2/3 of those were isolated increased inflammatory markers (19/32). Among major relapses, one resulted in permanent vision loss. The median time until relapse was 99 days (IQR 71–127) and median glucocorticoid dose at relapse was 8 mg (IQR 5–16). Nine of 47 patients stopped glucocorticoids after a median duration of 35 weeks and did not relapse within 1 year. Median CSD at 12 months was 4164 mg which is lower compared with published data. Glucocorticoid-associated adverse effects occurred in 40% of patients, most frequently were new onset or worsening hypertension (19%), diabetes (11%) and severe infections (11%). Conclusion We could demonstrate that 32% of patients remained relapse-free and 19% off glucocorticoids at 1 year after treatment with a rapid glucocorticoid tapering regimen. Most relapses were minor and could be handled with temporarily increased glucocorticoid doses. Consequently, the CSD at 12 months was much lower than reported in published cohorts. Thus, further reducing treatment-associated damage in patients with GCA by decreasing CSD seems to be possible.

Background The new ACR/EULAR 2022 classification criteria for giant cell arteritis (GCA) use weighted items and incorporate findings from vessel imaging to reflect the current clinical standard. Although intended to define homogenous patient populations for research purposes when a diagnosis of medium- or large-vessel vasculitis has been made, the 2022 criteria were developed including non-vasculitis comparators. Hence, in the absence of a sensitive diagnostic gold standard, the criteria bear potential to be used for GCA diagnosis. Objectives To evaluate the performance of the new 2022 ACR/EULAR classification criteria for GCA when used for GCA diagnosis. Methods Retrospective analysis of a cohort of patients suspected of having GCA who underwent ultrasound between 12/2006 and 05/2021. GCA was diagnosed if temporal artery biopsy was positive, if the 1990 ACR criteria were fulfilled, or if at least 2/5 ACR criteria were fulfilled in combination with vasculitis on imaging. Results 276 patients were diagnosed with GCA, and 400 patients had a condition that mimics GCA. When applying the 2022 criteria as diagnostic criteria, sensitivity remained high with 87.3% (95% CI 82.8%–91.0%), whereas specificity was lower with 70.3% (95% CI 65.5% to 74.7%). In 35 patients (12.7%) with GCA, the criteria were not met (Table 1). These were mainly patients without cranial symptoms but having typical imaging findings in vascular territories not considered in the criteria. 119 non-GCA patients (29.8%) scored ≥6 points. Polymyalgia rheumatica (PMR) (31.9%) followed by non-vasculitic ophthalmologic diseases (15.1%) were the most frequent diagnoses among those. Conclusion The inclusion of polymyalgic symptoms and heavy weighting of sudden visual loss in the score led to false classification of patients with PMR and ophthalmologic diseases as GCA. For diagnostic purposes, the scoring of the criteria could potentially be adapted by applying a higher cut-off for the diagnosis of GCA or a lower weighting for polymyalgic and visual symptoms. References [1]Ponte C, Grayson PC, Robson JC, et al. 2022 American College of Rheumatology/EULAR classification criteria for giant cell arteritis. Arthritis Rheumatol.2022. doi: 10.1002/art.42325. Acknowledgements The authors would like to thank the Swiss Foundation for Research on Muscle Diseases (FSRMM) for supporting the Ph.D. of Andrea Hemmig. Disclosure of Interests None Declared.Table 12022 American College of Rheumatology/ EULAR classification criteria for giant cell arteritis in patients with GCA and non-GCATotal number of patients (n=676)GCA patients with <6 points (n=35)GCA patients with ≥6 points (n=241)p-valueNon-GCA patients <6 points (n=281)Non-GCA patients ≥6 points (n=119)p-valueAge, mean (±SD)71.8 (9.7)71.3 (8.7)73.5 (8.4)0.16771.2 (10.2)70.2 (11.0)0.393Female, n (%)398 (58.9)21 (60.)150 (62.2)0.799155 (55.2)72 (60.5)0.324ACR/EULAR criteriaMorning stiffness in shoulders/neck, n (%)287 (42.5)8 (22.9)98 (40.7)0.004104 (37.0)77 (64.7)<0.001Sudden visual loss, n (%)78 (11.5)0 (0.0)36 (14.9)0.0121 (7.5)21 (17.6)0.002Jaw or tongue claudication, n (%)147 (21.7)0 (0.0)110 (45.6)<0.0019 (3.2)28 (23.5)<0.001New temporal headache, n (%)347 (51.3)11 (31.4)158 (65.6)<0.00184 (29.9)94 (79.0)<0.001Scalp tenderness, n (%)139 (20.6)1 (2.9)95 (39.4)<0.0019 (3.2)34 (28.6)<0.001Abnormal examination of the temporal artery, n (%)111 (16.4)1 (2.9)83 (34.4)<0.0017 (2.5)20 (16.8)<0.001Maximum ESR ≥50 mm/hour or maximum CRP ≥10 mg/liter, n (%)504 (74.6)29 (82.9)226 (93.8)0.004150 (53.4)99 (83.2)<0.001Positive temporal artery biopsy or halo sign on temporal artery ultrasound, n (%)194 (28.7)2 (5.7)186 (77.2)<0.0010 (0.0)6 (5.0)<0.001Bilateral axillary involvement, n (%)38 (5.6)2 (5.7)35 (14.5)0.1910 (0.0)1 (0.8)0.3FDG-PET activity throughout aorta, n (%)52 (7.7)5 (14.3)36 (14.9)0.9198 (2.8)3 (2.5)1CRP=C-reactive protein; ESR=erythrocyte sedimentation rate; FDG-PET=fluorodeoxyglucose-positron emission tomography; IQR=interquartile range; n=number; PMR=Polymyalgia rheumatica; SD=standard deviation; TA=temporal artery, TAB=temporal artery biopsy.

Background Subclinical giant cell arteritis (GCA) can be found in imaging studies at diagnosis of polymyalgia rheumatica (PMR) in up to 30% of patients [1]. Newly diagnosed GCA patients with a preceding PMR diagnosis may therefore present with more advanced vasculitic vessel alterations than GCA patients without prior PMR. Objectives To compare vascular ultrasound (US) findings in newly diagnosed GCA patients with and without a history of PMR. Methods Retrospective analysis of patients with GCA diagnosed at the University Hospital Basel between 12/2006 and 05/2021. Results 49 of 311 newly diagnosed GCA patients (15.8%) had a preceding diagnosis of PMR a median of 2.5 (IQR 0.6-5.6) years earlier. US revealed vessel wall alterations typical for vasculitis in the supraaortic vessels (carotid, vertebral, subclavian and/or axillary arteries) more often in patients with prior PMR than without PMR (51% vs 25%, p<0.001). Additionally, patients with prior PMR had more often significant vascular stenoses in the supraaortic vessels that were affected by vasculitis (37.5% vs 13.6%, p=0.03). Patients with and without prior PMR did not differ in terms of ischemic complications of stroke or vision loss at GCA diagnosis nor in the number of vascular segments with atherosclerosis (Table 1).In multivariable analysis, prior PMR was significantly associated with vasculitic vessel wall alterations (OR 4.28, 95% CI 1.92–9.85, p<0.001) adjusted for sex, headache, scalp and temporal artery tenderness. Furthermore, prior PMR was associated with vascular stenoses (4.97, 95% CI 1.4–19.12, p=0.015) adjusted for age, sex, arteriosclerosis, cerebrovascular and coronary heart disease. Conclusion GCA patients with prior PMR had a more extensive vascular involvement on ultrasound than those without prior PMR at diagnosis. Presence of stenoses could not be explained by arteriosclerosis. Undiagnosed subclinical GCA in patients with previous PMR may be a cause of more advanced vascular changes. These data support the need for screening strategies for GCA in patients with PMR. Reference [1]Hemmig AK, Gozzoli D, Werlen L, et al. Subclinical giant cell arteritis in new onset polymyalgia rheumatica A systematic review and meta-analysis of individual patient data. Semin Arthritis Rheum. 2022. 28;55:152017. Acknowledgements The authors would like to thank the Swiss Foundation for Research on Muscle Diseases (FSRMM) for supporting the Ph.D. of Andrea Hemmig. Disclosure of Interests Andrea Hemmig: None declared, Markus Aschwanden: None declared, Christoph Berger: None declared, Diego Kyburz Consultant of: AbbVie, Eli Lilly, Janssen, Novartis, Pfizer, Roche, Grant/research support from: Abbvie, Noemi Mensch: None declared, Daniel Staub: None declared, Mihaela Stegert: None declared, Stephan Imfeld: None declared, Thomas Daikeler: None declared.Table 1Patient characteristicsCharacteristicsAll (n=311)GCA without history of PMR (n=262)GCA with history of PMR (n=49)p-valueAge (years), mean±SD73.8 (7.6-78.8)73.5 (67.3-79.2)74.4 (70.0-78.0)0.678Female196 (63.0)166 (63.4)30 (61.2)0.776Hypertension156 (50.2)128 (48.9)28 (57.1)0.301Diabetes mellitus57 (18.3)49 (18.7)8 (16.3)0.733Dyslipidemia78 (25.1)64 (24.4)14 (28.6)0.539Smoking101 (32.5)91 (34.7)10 (20.4)0.038Arteriosclerotic segments*1 (0.0-2.0)1 (0.0-2.0)0.0 (0.0-2.0)0.078Coronary artery disease44 (14.1)39 (14.9)5 (10.2)0.354Cerebrovascular disease29 (9.3)22 (8.4)7 (14.3)0.282ESR (mm/h)70 (40.0-88.0)72 (41.5-90.0)50.0 (28.0-72.0)0.002CRP (mg/dl)55.7 (24.1-109.3)57.6 (25.6-110.3)52.8 (17.3-102.5)0.336Headache188 (60.5)164 (62.6)24 (49.0)0.091Jaw claudication126 (40.5)110 (42.0)16 (32.7)0.3Scalp tenderness112 (36.0)102 (38.9)10 (20.4)0.02Polymyalgic symptoms119 (38.3)94 (35.9)25 (51.0)0.053Stroke14 (4.5)10 (3.8)4 (8.2)0.249Permanent vision loss45 (14.5)38 (14.5)7 (14.3)0.968Vasculitis in supraaortic vessels**90 (28.9)65 (24.8)25 (51.0)<0.001Vascular stenoses (≥50%)†17/83 (20.5)8/59 (13.6)9/24 (37.5)0.032n (%); median (interquartile range) for numeric values.CRP: C-reactive protein, ESR: erythrocyte sedimentation rate, GCA: giant cell arteritis, PMR: polymyalgia rheumatica.*Number of vascular segments with atherosclerosis on ultrasound examination in both carotid, vertebral, subclavian and axillary arteries.**Number of patients with vasculitis in the carotid, vertebral, subclavian and/or axillary arteries.†Number of patients with vascular stenoses in the internal carotid, axillary and/or vertebral arteries affected by vasculitis (n=83).

Background: We sought to investigate magnetic resonance imaging (MRI) parameters that correspond to vasculitis observed via [18F]FDG positron emission tomography/computed tomography (PET/CT) and ultrasound in patients with large-vessel giant cell arteritis (LV-GCA). Methods: We performed a cross-sectional analysis of patients diagnosed with LV-GCA. Patients were selected if MRI, PET/CT, and vascular ultrasound were performed at the time of LV-GCA diagnosis. Imaging findings in vessel segments (axillary segment per side, thoracic aorta) assessed using at least two methods were compared. Vessel wall thickening, oedema, and contrast agent enhancement were each assessed via MRI. Results: Twelve patients with newly diagnosed LV-GCA were included (seven females, 58%; median age 72.1, IQR 65.5–74.2 years). The MRI results showed mural thickening in 9/24 axillary artery segments. All but 1 segment showed concomitant oedema, and additional contrast enhancement was found in 3/9 segments. In total, 8 of these 9 segments corresponded to vasculitic findings in the respective segments as observed via PET/CT, and 2/9 corresponded to vasculitis in the respective ultrasound images. If MRI was performed more than 6 days after starting prednisone treatment, thickening and oedema were seen in only 1/24 segments, which was also pathologic according to ultrasound findings but not those obtained via PET/CT. Four patients had mural thickening, oedema, and contrast enhancement in the aorta, among whom three patients also had vasculitic findings observed via PET/CT. Isolated mural thickening in one patient corresponded to a negative PET/CT result. Conclusions: In the MRI results, mural thickening due to oedema corresponded to vasculitic PET/CT findings but not vasculitic ultrasound findings. The duration of steroid treatment may reduce the sensitivity of MRI.

Objectives To characterise factors associated with permanent vision loss (PVL) and potential reasons for the therapeutic delay contributing to PVL in giant cell arteritis (GCA). Methods Retrospective analysis of GCA patients diagnosed at the University Hospital Basel between December 2006 and May 2021. Results Of 282 patients with GCA (64% females), 49 (17.4%) experienced PVL. In 43/49 (87.8%) PVL occurred before treatment. Of these, 24 (55.8%) patients had first non-ocular symptoms and eventually sought consultation when PVL occurred in a median of 21 (IQR 14.75–31.0) days after the first symptoms. Only five of the 24 patients had consulted a physician before PVL, but GCA diagnosis was missed. Treatment was initiated rapidly after diagnosis (median 1 day (IQR 0.0–7.0)). PVL on therapy occurred in six patients in a median of 40 (IQR 20.5–67.3) days after treatment started. In two of those, glucocorticoids were tapered too quickly. In multivariable analysis, patients with PVL were older (OR 1.17, 95% CI 1.07 to 1.29, p=0.001) and reported more frequently jaw claudication (OR 3.52, 95% CI 1.02 to 13.16, p=0.051). PVL was present in 18 (42.9%) of the 42 patients with vasculitic ultrasound findings in all six temporal artery segments. The incidence of PVL over 15 years did not decline (Spearman rank=0.3, p=0.68). Conclusion The prevalence of GCA-associated PVL remains high. Associated factors were advanced age, jaw claudication and ultrasound findings consistent with vasculitis in all six temporal artery segments. Despite preceding non-ocular GCA symptoms weeks before the onset of PVL, most patients were not seen by a rheumatologist before PVL occurred.

We previously proposed standard uptake value (SUV) ratio-based cut-off values for [18F] fluorodeoxyglucose-positron emission tomography/computed tomography (PET/CT) for diagnosing giant cell arteritis (GCA) with high diagnostic accuracy. Here we confirm our findings in an independent cohort and report a simplified procedure for using a SUV ratio to diagnose LV-GCA. Patients with suspected GCA were consecutively included. The ‘peak SUV ratio’ was defined in a two-step approach. First, the vessel with the visually brightest radiotracer uptake in the supra-aortic (SA) and in the aorto-iliofemoral (AIF) region was identified. Here, the maximum SUV of the vessel was measured and divided by the mean SUV of the liver (SUVratio). A ratio >1.0 in the SA or >1.3 in the AIF region was scored as vasculitis. The diagnostic accuracy, sensitivity, and specificity of the ‘peak SUV ratio’ in the SA and AIF region was assessed. From 2015 to 2019, 50 patients (24 female, median age 71 years) with suspicion of GCA were included, 28 patients with GCA and 22 patients with exclusion of GCA. Peak SUV had an AUC of 0.91, a sensitivity of 0.89, and a specificity of 0.73 for diagnosing GCA. Peak SUV accuracy of the AIF arteries was lower (AUC 0.81) than of the SA arteries (AUC 0.95). Our SUV ratio cut-off values for diagnosing GCA are consistently valid, also when applied in a time-efficient clinical procedure focusing on the peak SUV ratio. The diagnostic performance of PET/CT in this validation cohort was even higher, compared to the inception cohort (AUC of 0.83).

Introduction The purpose of this article is to report on the implementation of a telemedicine network serving as a second opinion pool for a surgeon in a remote area of a developing country. Methods This study involved an international collaboration between two members of Swiss Surgical Teams at a tertiary referral hospital and a surgeon in a remote area in Gorno-Badakhshan Autonomic Oblast, Tajikistan, which established a second opinion pool discussing diagnostics and therapeutic options via a messenger application. A retrospective analysis of response times was performed using a series of 50 challenging cases. Results The median time to receive a first telemedical response from any of the two contacts was 24 min (interquartile range 6–73). Urgent and emergent pathologies accounted for 57% of cases. The suggested treatment was carried out in 90% ( n = 44) of cases. Conclusions Timely and convenient telemedicine support to provide diagnostic and therapeutic reassurance and improve treatment quality for patients presenting to a general and vascular surgeon in the large and remote region of Gorno-Badakhshan Autonomic Oblast can be installed via a messenger application.

OBJECTIVES:To determine the prevalence and predictors of subclinical giant cell arteritis (GCA) in patients with newly diagnosed polymyalgia rheumatica (PMR).METHODS:PubMed, Embase, and Web of Science Core Collection were systematically searched (date of last search July 14, 2021) for any published information on any consecutively recruited cohort reporting the prevalence of GCA in steroid-naïve patients with PMR without cranial or ischemic symptoms. We combined prevalences across populations in a random-effect meta-analysis. Potential predictors of subclinical GCA were identified by mixed-effect logistic regression using individual patient data (IPD) from cohorts screened with PET/(CT).RESULTS:We included 13 cohorts with 566 patients from studies published between 1965 to 2020. Subclinical GCA was diagnosed by temporal artery biopsy in three studies, ultrasound in three studies, and PET/(CT) in seven studies. The pooled prevalence of subclinical GCA across all studies was 23% (95% CI 14%-36%, I2=84%) for any screening method and 29% in the studies using PET/(CT) (95% CI 13%-53%, I2=85%) (n=266 patients). For seven cohorts we obtained IPD for 243 patients screened with PET/(CT). Inflammatory back pain (OR 2.73, 1.32-5.64), absence of lower limb pain (OR 2.35, 1.05-5.26), female sex (OR 2.31, 1.17-4.58), temperature >37° (OR 1.83, 0.90-3.71), weight loss (OR 1.83, 0.96-3.51), thrombocyte count (OR 1.51, 1.05-2.18), and haemoglobin level (OR 0.80, 0.64-1.00) were most strongly associated with subclinical GCA in the univariable analysis but not C-reactive protein (OR 1.00, 1.00-1.01) or erythrocyte sedimentation rate (OR 1.01, 1.00-1.02). A prediction model calculated from these variables had an area under the curve of 0.66 (95% CI 0.55-0.75).CONCLUSION:More than a quarter of patients with PMR may have subclinical GCA. The prediction model from the most extensive IPD set has only modest diagnostic accuracy. Hence, a paradigm shift in the assessment of PMR patients in favour of implementing imaging studies should be discussed.

BACKGROUND: It is not known what diagnoses are associated with an elevated D-dimer in unselected patients attending emergency departments (ED), nor have their associated outcomes been determined. METHODS: This was a prospective observational study of 1612 unselected patients attending a Danish ED, with 100% follow-up for 90 days after presentation. RESULTS: The 765 (47%) ED patients with an elevated D-dimer level (ie, >= 0.5 mg/L) were more likely to be admitted to hospital (P < .0001), re-present to health services (P = .02), and die within 90 days (8.1% of patients, P < .0001). Only 10 patients with a normal D-dimer level (1.2%) died within 90 days. Five had chronic obstructive pulmonary disease and infection, and 5 had cancer (4 of whom also had infection). Venous thromboembolism, infection, neoplasia, anemia, heart failure, and unspecified soft tissue disorders were significantly associated with an elevated D-dimer level. Of the 72 patients with venous thromboembolism, 20 also had infection, 8 had cancer, and 4 had anemia. None of the patients with heart failure, stroke, or acute myocardial infarction with a normal D-dimer level died within 90 days. CONCLUSIONS: In this study, nearly half of all patients attending the ED had an elevated D-dimer level, and these patients were more likely to be admitted to hospital and to re-present to health services or die within 90 days. In this unselected ED patient population, elevated D-dimer levels were found to not only be significantly associated with venous thromboembolism, but to also be associated with infection, cancer, heart failure, and anemia. (C) 2020 Elsevier Inc. All rights reserved.

BACKGROUND AND AIM Acute decompensation and death have been observed in patients with acute hepatitis E virus (HEV) infection and preexisting liver cirrhosis. However, the clinical, laboratory and histological features need to be fully characterised. METHODS Some of us recently described the histological presentation of hepatitis E in a large panel of liver tissue specimens. Here, we conducted a case-control study to investigate the clinical and laboratory features of the subset of patients with HEV-related acute-on-chronic liver failure (ACLF) and death. Each patient was matched to three control patients with histologically confirmed severe alcoholic hepatitis based on sex, age, total bilirubin, INR, serum creatinine and MELD score on admission. RESULTS Of 5 patients who died in a context of HEV-related ACLF, 3 (60%) were male and the median age was 66 years (range 51–76). Median alanine aminotransferase (ALT) at presentation was 2610 U/l (range 705–3134) and aspartate aminotransferase (AST) 2818 U/l (range 1176–8611). Liver function was heavily altered in all patients. Histological analyses revealed steatohepatitis on a background of cirrhosis, suggestive of an alcoholic or nonalcoholic origin. Based on histopathology, alcoholic hepatitis was initially suspected in two patients and corticosteroid treatment was initiated. Ribavirin was started in four patients. Median time from hospitalisation to death was 17 days (range 6–25 days). AST levels in patients with HEV-related ACLF were significantly higher as compared to the matched patients with severe alcoholic hepatitis. CONCLUSION Typical histopathological features of viral hepatitis may be absent in ACLF caused by HEV infection. HEV infection should be sought in acute decompensation of cirrhosis and ACLF even in the absence of histological changes suggesting viral infection.

Background:GCA is characterized by cranial symptoms but imaging techniques show that patients with non-specific symptoms such as systemic inflammation or PMR may have undiagnosed large vessel (LV) GCA1. Although silent GCA in patients with clinically isolated PMR may have consequences for patients’ outcome, little is known about its prevalence and characteristics of affected patients.Objectives:To review data on the prevalence of silent GCA in newly diagnosed PMR patients without cranial GCA symptoms and to analyze which characteristics are associated with vascular involvement among PMR patients.Methods:We systematically screened PubMed, Embase and Web of Science databases and included studies screening for GCA in steroid naïve PMR patients without cranial symptoms consistent with GCA. Authors of the publications that used PET for vasculitis screening were invited to share their individual patient data (IPD) for a meta-analysis. We sought to define patient characteristics that were associated with vasculitis using univariable mixed effects logistic regression models with vascular involvement as the outcome, missing values were imputed using multilevel joint modeling multiple imputation. To fit a multivariable model with the candidate predictors we excluded variables that were hypothesized to have less medical relevance for the outcome and highly correlated inflammation markers (ESR, Lc).Results:Out of the 3047 studies screened independently by 2 authors (DG and TD), 13 fulfilled the inclusion criteria. These studies (published 1963-2019) reported on 543 PMR patients examined by temporal artery biopsy (n=175), ultrasound (n=110), PET or PET-CT (n=258). 115 PMR patients were diagnosed with GCA (21.2%), with prevalence ranging from 0-92%.We collected IPD for 243 patients from 4 cohorts using PET and 3 using PET/CT for GCA diagnosis. The overall median age of patients was 72.3 years (IQR 66.4-78.0) and vasculitis was found in 65 patients (26.7%) (table 1).Table 1.OverallPMRPMR+GCAn (%)243178 (73.3)65 (26.7)Female sex (%)146 (60.1)98 (55.1)48 (73.8)Shoulder girdle pain (%)236 (97.1)174 (97.8)62 (95.4)Pelvic girdle pain (%)174 (71.6)127 (71.3)47 (72.3)Inflammatory back pain (%)No107 (44.0)83 (46.6)24 (36.9)Yes106 (43.6)70 (39.3)36 (55.4)Lower limb pain (%)No87 (35.8)61 (34.3)26 (40.0)Yes81 (33.3)68 (38.2)13 (20.0)Weight loss (%)112 (46.1)78 (43.8)34 (52.3)CRP (mg/l) (median [IQR])46.0 [19.0, 77.7]44.0 [16.9, 74.2]52.0 [27.9, 85.0]ESR (mm/h) (mean (SD))65.2 (30.3)62.7 (30.2)72.3 (29.7)Hemoglobin (g/dl) (mean (SD))12.1 (1.5)12.2 (1.5)11.7 (1.6)Thrombocytes (1e+09/ml) (mean (SD))341.9 (106.3)323.9 (103.2)375.8 (104.6)In the univariable analyses the following factors were most strongly associated with vasculitic PET findings: female sex (OR 2.31, CI 1.17-4.58), inflammatory back pain (OR 2.73, CI 1.32-5.64), temperature >37° (OR 1.83, CI 0.90-3.7), weight loss (OR 1.83, CI 0.96-3.51), thrombocytosis (i.e., patients with a thrombocyte count 1 SD above mean have an OR of 1.51, CI 1.05-2.18), anemia (i.e., 1 g/dl decrease in Hb below mean corresponds to an OR of 1.25, CI 1.00-1.56). Patients with lower limb pain were less likely to have vasculitis (OR 0.43, CI 0.19–0.95). The estimated ORs were very similar in the multivariable model although the 95%CIs became wider.Conclusion:Although the prevalence across published studies showed substantial variation, 6 out of 13 studies reported a prevalence of silent GCA in 18-40% of all PMR patients. The exploratory analysis of the collected IPD identified female sex, inflammatory back pain, fever, weight loss, absence of lower leg pain, thrombocytosis and anemia as factors associated with LV-GCA. These findings should be validated in future prospective cohort studies. The presence or absence of these factors may further aid in diagnosing LV-GCA in PMR patients.References:[1]Buttgereit F, Dejaco C, Matteson EL, Dasgupta B. Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review. JAMA. 2016 Jun 14;315(22):2442–58.Acknowledgements:The study is funded by the “Schweizerische Stiftung für die Erforschung der Muskelkrankheiten (SSEM)”.Disclosure of Interests:Daniele Silvio Gozzoli: None declared, Andrea Hemmig: None declared, Lars Hemkens: None declared, Laura Werlen: None declared, Hannah Ewald: None declared, Christoph Berger: None declared, Diego Kyburz Grant/research support from: DK reports personal fees from Abbvie, Gilead, Lilly, Novartis and Pfizer, outside of the submitted work, Stephan Imfeld: None declared, Markus Aschwanden: None declared, Mihaela Stegert: None declared, Dario Camellino: None declared, Marco Amedeo Cimmino: None declared, Corrado Campochiaro Grant/research support from: personal fees from Roche, Alessandro Tomelleri: None declared, Liesbet Henckaerts: None declared, Daniel Blockmans Speakers bureau: Paid speaker for Roche, Consultant of: Paid consultant for Roche, Patricia Moya: None declared, Hector Corominas: None declared, Russell Buchanan: None declared, Claire Owen Speakers bureau: CO has received speaking honoraria from Roche, Janssen, Novartis and Pfizer, and meeting sponsorship from Roche, UCB and Janssen, Yannick van Sleen: None declared, Elisabeth Brouwer Speakers bureau: E. Brouwer as an employee of the UMCG received speaker fees and consulting fees from Roche in 2017, 2018 which were paid to the UMCG, Consultant of: E. Brouwer as an employee of the UMCG received speaker fees and consulting fees from Roche in 2017, 2018 which were paid to the UMCG, Hiroyuki Ymashita: None declared, Thomas Daikeler: None declared

Background: For the diagnosis of giant cell arteritis (GCA) several diagnostic tools do exist such as 18F-FDG-PET/CT (PET) with excellent diagnostic accuracy for the larger vessels and ultrasound for the temporal arteries (TA). Recent data propose that PET is able to detect vasculitis in vessels as small as the TA (1). Comparison of PET, ultrasound (US) and histology of the TA on a segment level has not been done. Objectives: To describe diagnostic accuracy of PET of the TA in a vasculitis university clinic and to analyse strength and limitations of PET by comparing 18F-FDG uptake to US and histology results on a segment level. Methods: We analysed patients, included in our ethical board approved local prospective GCA cohort having received a PET in between 2015 and 2019 because of suspected GCA. PET of the TA was performed using time-of flight technique and was scored ‘vasculitis’ if tracer uptake was higher than in the surrounding tissue. Standard uptake value (SUV) measurement in the trunk (T), parietal branch (PB) and frontal branch (FB) of the TA was recorded. US was performed for each branch. Results: From 37 consecutively recruited patients, GCA was confirmed in 19 patients and excluded in 18 patients which served as controls (Table 1). PET of the TA showed vasculitis in 12/19 GCA patients and in 1/18 controls. Median SUVmax of all vasculitic FB (n=18) was 2.91, 2.20 for the T (n=14) and 2.34 for the PB (n=5). 16 of the 19 GCA patients received US of the TA and 9 showed vasculitic findings. From the control group 2 patients showed vasculitic findings. Most often vasculitic findings were localized in the FB (n=16), followed by the T (n=13) and the PB (n=13). In the 16 patients that received US, diagnostic sensitivity and specificity of temporal PET for GCA within the TA was 56% and 94% and of US 56% and 89%, respectively. Whereas US detects vasculitis in comparable frequencies in all TA branches, PET recorded vasculitis less often in the PB (only 4 of the 13 in US vasculitic PB). Indeed, the median diameter of all PET positive TA branches, measured in the US, was higher (2.00mm) compared to PET negative branches (1.50mm). Vasculitis was confirmed histologically in 9 of the 13 biopsied patients. Only 2/9 patients showed vasculitis in the preceding PET in the biopsied branch. Conclusion: High diagnostic accuracy for temporal arteries supports PET as an ‘all-in-one’ exam for GCA. A limitation might be the vessel diameter, as sensitivity of PET for vasculitis of the small parietal branch is low. Thus, in cases with high suspicion of GCA despite a negative PET, US of the TA and or biopsy might enhance diagnostic sensitivity. References: [1]Nielsen et al. Simple dichotomous assessment of cranial artery inflammation by conventional 18F-FDG PET/CT shows high accuracy for the diagnosis of giant cell arteritis: a case-control study. Eur J Nucl Med Mol Imaging. 2019;46(1):184-193. doi:10.1007/s00259-018-4106-0 Disclosure of Interests: None declared

Background: For the diagnosis of giant cell arteritis (GCA) several diagnostic tools do exist such as 18 F-FDG-PET/CT (PET) with excellent diagnostic accuracy for the larger vessels and ultrasound for the temporal arteries (TA). Recent data propose that PET is able to detect vasculitis in vessels as small as the TA (1). Comparison of PET, ultrasound (US) and histology of the TA on a segment level has not been done. Objectives: To describe diagnostic accuracy of PET of the TA in a vasculitis university clinic and to analyse strength and limitations of PET by comparing 18 F-FDG uptake to US and histology results on a segment level. Methods: We analysed patients, included in our ethical board approved local prospective GCA cohort having received a PET in between 2015 and 2019 because of suspected GCA. PET of the TA was performed using time-of flight technique and was scored ‘vasculitis’ if tracer uptake was higher than in the surrounding tissue. Standard uptake value (SUV) measurement in the trunk (T), parietal branch (PB) and frontal branch (FB) of the TA was recorded. US was performed for each branch. Results: From 37 consecutively recruited patients, GCA was confirmed in 19 patients and excluded in 18 patients which served as controls (Table 1). PET of the TA showed vasculitis in 12/19 GCA patients and in 1/18 controls. Median SUV max of all vasculitic FB (n=18) was 2.91, 2.20 for the T (n=14) and 2.34 for the PB (n=5). Table 1. Patient characteristics. Data are expressed as number (%) or median (interquartile range) GCA (n=19 ) (51% ) Control (n=18 ) (49% ) P-value Female 11 (57) 9 (50) 0.64 Median age (years) at PET 73 (64-78) 62.5 (57-71.75) 0.04 Amaurosis fugax/Loss of vision 9 (47) 9 (50) >0.99 New onset headache 13 (68) 11 (61) 0.74 Jaw claudication 7 (37) 3 (16) 0.38 Scalp tenderness/ pathological TA 7 (37) 6 (33) 0.54 Proximal muscle pain 11 (58) 8 (44) 0.29 Fever 1 (5) 5 (28) 0.06 Median Erythrocyte sedimentation rate (mm/h) 73 (48-90) 50 (28.5 - 68.5) 0.06 Median C-reactive protein (mg/L) 66 (29-105) 46 (13-133) 0.57 16 of the 19 GCA patients received US of the TA and 9 showed vasculitic findings. From the control group 2 patients showed vasculitic findings. Most often vasculitic findings were localized in the FB (n=16), followed by the T (n=13) and the PB (n=13). In the 16 patients that received US, diagnostic sensitivity and specificity of temporal PET for GCA within the TA was 56% and 94% and of US 56% and 89%, respectively. Whereas US detects vasculitis in comparable frequencies in all TA branches, PET recorded vasculitis less often in the PB (only 4 of the 13 in US vasculitic PB). Indeed, the median diameter of all PET positive TA branches, measured in the US, was higher (2.00mm) compared to PET negative branches (1.50mm). Vasculitis was confirmed histologically in 9 of the 13 biopsied patients. Only 2/9 patients showed vasculitis in the preceding PET in the biopsied branch. Conclusion: High diagnostic accuracy for temporal arteries supports PET as an ‘all-in-one’ exam for GCA. A limitation might be the vessel diameter, as sensitivity of PET for vasculitis of the small parietal branch is low. Thus, in cases with high suspicion of GCA despite a negative PET, US of the TA and or biopsy might enhance diagnostic sensitivity. References: [1]Nielsen et al. Simple dichotomous assessment of cranial artery inflammation by conventional 18F-FDG PET/CT shows high accuracy for the diagnosis of giant cell arteritis: a case-control study. Eur J Nucl Med Mol Imaging . 2019;46(1):184-193. doi: 10.1007/s00259-018-4106-0 Disclosure of Interests: None declared

Objectives: [18F]Fluorodeoxyglucose (FDG)-PET/CT and US are both well established for diagnosing GCA. The present study investigates their accuracy and whether they provide overlapping or complementary information in a cohort of patients presenting with suspicion of GCA. Methods: We selected consecutive patients from our cohort of suspected GCA cases that underwent both extended vascular US and PET/CT for diagnostic work-up between December 2006 and August 2012. Results: A total of 102 patients were included. Diagnosis of GCA was confirmed in 68 patients and excluded in 34 patients (controls). Vasculitic changes in US were most often found in the temporal artery with 32 positive findings on each side, followed by the popliteal artery (10 right, 9 left) and the subclavian/axillary artery (7 right, 8 left). By contrast, PET/CT showed vasculitis most frequently in the vertebral (23 right, 33 left) and common carotid arteries (32 right, 24 left), followed by the subclavian arteries (16 right, 18 left), and the thoracic (17) and abdominal aorta (23). In 37/68 GCA patients PET/CT and US both revealed vasculitic findings, 11/68 had positive findings in US only and 14/68 in PET/CT only. Specificity of US was higher (one false-positive vs five false-positive in PET/CT). On a single segment level, only 20 of 136 positive segments were positive in both imaging modalities. Conclusion: PET/CT measuring vessel wall metabolism and US vessel wall morphology showed a comparable diagnostic accuracy for GCA. However PET/CT and US were often discrepant within single vascular regions. Thus PET/CT and US should be considered as complementary methods, with a second imaging modality increasing the diagnostic yield by 16-20%.