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

Objective: Optic nerve sheath enhancement on magnetic resonance imaging has been reported in patients with giant cell arteritis (GCA), with or without visual manifestations. Whether similar findings can be documented on ultrasound is unknown. Optic nerve ultrasound is a point‐of‐care, easy to learn, rapid, and noninvasive technique. This study aims to investigate whether optic nerve sheath diameter (ONSD) measured on ultrasound is useful in the diagnosis of active, new‐onset GCA. Methods: A single‐center, diagnostic accuracy study was performed from June to November 2022 on consecutive eligible patients referred for suspected GCA. Optic nerve ultrasound was performed on both eyes. The ONSD (includes the optic nerve and its sheath) and optic nerve diameter (OND) were measured 3 mm behind the ocular globe. The presence or absence of GCA was confirmed clinically 6 months later. Multivariable linear regression, adjusting for age and sex, was used to determine the association between optic nerve ultrasound measures and final GCA diagnosis. Results: Thirty participants were enrolled, including nine participants with a final diagnosis of GCA. Mean ± SD ONSD was 5.98 ± 1.17 mm in patients with GCA and 4.02 ± 0.99 mm in patients without GCA. Mean ONSD was greater by 1.26 mm in patients with GCA (95% confidence interval 0.30–2.21 mm, P = 0.01) compared with those without GCA, adjusting for age and sex. Mean ± SD OND was 2.97 ± 0.46 mm in patients with GCA and 2.47 ± 0.58 mm in patients without GCA. There was no evidence of an association between GCA diagnosis and OND. Conclusion: Patients with GCA had a significantly greater ONSD on ultrasound than patients without GCA. Optic nerve ultrasound may represent a novel, rapid, bedside diagnostic test for GCA. A large prospective study is required to confirm these findings and evaluate whether ONSD can be used as a disease activity biomarker in GCA. [ABSTRACT FROM AUTHOR]

Objectives 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. Methods 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. Results 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%. Conclusions 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. [ABSTRACT FROM AUTHOR]

The article discusses research which focused on non-specific symptoms pathway (NSSP) programmes and the diagnosis of large vessel giant cell arteritis (LV-GCA). Topics explored include the use of the NSSP programme by primary care physicians in making referrals for patients with possible malignancy, the common clinical manifestations of LV-GCA, and the diagnostic accuracy of computed tomography (CT) imaging in identifying LV-GCA.

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. [ABSTRACT FROM AUTHOR]

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

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

Objective: It is urgent to diagnose giant cell arteritis (GCA) as quickly as possible to prevent irreversible blindness. The traditional gold standard for diagnosing GCA is temporal artery biopsy (TAB). However, TAB lacks diagnostic performance and carries out risks of surgical intervention. The noninvasive color Doppler ultrasound (CDU) seems to be a promising alternative. This study is designed to assess the diagnostic value of CDU in daily clinical practice. Methods: In this prospective cohort study, patients with a clinical suspicion of active GCA were included and underwent a CDU of the temporal arteries. If deemed necessary by the referrer, a TAB and/or 18F‐fluorodeoxyglucose positron emission tomography with computed tomography was performed. The retrospective clinical diagnosis was determined 1 year after inclusion by two physicians experienced in the field of vasculitis. Results: 242 patients were included and GCA was diagnosed in 73 (30%) patients by the defined retrospective clinical diagnosis. Compared with the retrospective diagnosis, CDU has a sensitivity of 60% (48–72), specificity of 94% (89–97), positive predictive value (PPV) of 81% (70–89), negative predictive value (NPV) of 85% (80–88), and an accuracy of 84% (78–88). A total of 84 (35%) patients underwent TAB. TAB has a sensitivity of 66% (51–79), specificity of 100% (90–100), PPV of 100% (100), NPV of 67% (58–75), and an accuracy of 80% (70–88). Conclusion: This study shows comparable diagnostic performance for CDU and TAB and even better CDU results with a bilateral halo present. Considering the advantages of the noninvasive CDU, it is the diagnostic tool of choice. [ABSTRACT FROM AUTHOR]

Background: The aim of this work is to develop guidelines for health care professionals in the giant cell arteritis diagnosis and management, based on patients' stratification and targeted outcome measures. Results: Fourteen expert panel had completed the two rounds surveys. After the end of round two, twenty three recommendations were released distributed on 8 domains. The percentage of the agreement on the recommendations was 76.9% to 100%. All 23 key questions were answered at the end of the second round with agreement upon. Conclusion: Patient stratification facilitate the initiation of an appropriate management approach for patients with giant cell arteritis aiming at achieving targeted disease remission state and prevention of visual loss and/or development of ischaemic events. Treat to Target approach is a new concept in giant cell arteritis management which aims to provide tight control to achieve and maintain disease remission. This work defined the treatment targets in relation to the disease stage. [ABSTRACT FROM AUTHOR]

Objective The main objective of this study was to analyse the prevalence and characteristics of subclinical GCA in patients with PMR. Methods This was a cross-sectional multicentre international study of consecutive patients with newly diagnosed PMR without symptoms or signs suggestive of GCA. All patients underwent US of the temporal superficial, common carotid, subclavian and axillary arteries. Patients with halo signs in at least one examined artery were considered to have subclinical GCA. The clinical, demographic and laboratory characteristics of the PMR group without subclinical vasculitis were compared with subclinical GCA, and the pattern of vessel involvement was compared with that of a classical single-centre GCA cohort. Results We included 346 PMR patients, 267 (77.2%) without subclinical GCA and 79 (22.8%) with subclinical GCA. The PMR patients with subclinical GCA were significantly older, had a longer duration of morning stiffness and more frequently reported hip pain than PMR without subclinical GCA. PMR with subclinical GCA showed a predominant extracranial large vessel pattern of vasculitic involvement compared with classical GCA, where the cranial phenotype predominated. The patients with PMR in the classical GCA group showed a pattern of vessel involvement similar to classical GCA without PMR but different from PMR with subclinical involvement. Conclusion More than a fifth of the pure PMR patients had US findings consistent with subclinical GCA. This specific subset of patients showed a predilection for extracranial artery involvement. The optimal screening strategy to assess the presence of vasculitis in PMR remains to be determined. [ABSTRACT FROM AUTHOR]

The article describes a case of an older male patient with genetic history of transthyretin amyloidosis. Topics mentioned include the similarity of the disease with giant cell arteritis, the presence of ill-defined erythematous rash on the face with ecchymosis, the results of the routine blood tests, and the clinical manifestations of the amyloidosis.

The article presents a study which questioned whether clonal haematopoiesis (CH)-associated gene mutations, including UBA1 mutations, contribute to the pathogenesis of giant cell arteritis (GCA) and evaluated the clonal expansion of CH over time. Topics include the potential contribution of CH to the pathogenesis of inflammatory diseases, annual incidence rate of biopsy-proven GCA, and prevalence of CH in individuals with GCA from a population-based cohort.

Giant cell arteritis (GCA) is a granulomatous arteritis involving large arteries, particularly the aorta and its major proximal branches, including the carotid and temporal arteries. GCA involves individuals over 50 years old. The etiopathogenesis of GCA may involve a genetic background triggered by unknown environmental factors (eg infections), the activation of dendritic cells as well as inflammatory and vascular remodeling. However, its pathogenetic mechanism still remains unclear, although progress has been made in recent years. In the past, inflammatory markers and arterial biopsy were considered as gold standard for the diagnosis of GCA. However, emerging imaging methods have been made more sensitive and specific for the diagnosis of GCA. Treatment includes biological and other modalities including interleukin-6 (IL-6) inhibitors. [ABSTRACT FROM AUTHOR]

Objectives To explore current management practices for PMR by general practitioners (GPs) and rheumatologists including implications for clinical trial recruitment. Methods An English language questionnaire was constructed by a working group of rheumatologists and GPs from six countries. The questionnaire focused on: 1: Respondent characteristics; 2: Referral practices; 3: Treatment with glucocorticoids; 4: Diagnostics; 5: Comorbidities; and 6: Barriers to research. The questionnaire was distributed to rheumatologists and GPs worldwide via members of the International PMR/Giant Cell Arteritis Study Group. Results In total, 394 GPs and 937 rheumatologists responded to the survey. GPs referred a median of 25% of their suspected PMR patients for diagnosis and 50% of these were returned to their GP for management. In general, 39% of rheumatologists evaluated patients with suspected PMR >2 weeks after referral, and a median of 50% of patients had started prednisolone before rheumatologist evaluation. Direct comparison of initial treatment showed that the percentage prescribing >25 mg prednisolone daily for patients was 30% for GPs and 12% for rheumatologists. Diagnostic imaging was rarely used. More than half (56%) of rheumatologists experienced difficulties recruiting people with PMR to clinical trials. Conclusion This large international survey indicates that a large proportion of people with PMR are not referred for diagnosis, and that the proportion of treatment-naive patients declined with increasing time from referral to assessment. Strategies are needed to change referral and management of people with PMR, to improve clinical practice and facilitate recruitment to clinical trials. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) is the most common form of vasculitis present in adults. Its symptoms result from ischemia of the areas supplied by the arteries or the severity of the inflammatory reaction: headache, jaw and limb claudication, visual disturbances, blindness, stroke, polymyalgia, and fever. Because of the variety of symptoms, the disease is often overlooked in diagnostics, possibly leading to permanent ischemic complications. The current classification criteria and the gold standard for diagnostics - temporal artery biopsy - apply to the cranial form of the disease. European Alliance of Associations for Rheumatology guidelines have systematized diagnostics, based mainly on simple and reproducible ultrasound examination (ultrasonography). Despite the widespread availability of this imaging method, GCA is still diagnosed too late, and therefore the authors analyzed the possible diagnostic difficulties, based on a group of 21 patients. [ABSTRACT FROM AUTHOR]

Objectives Temporal artery magnetic resonance angiography (TAMRA) is a useful tool to investigate possible diagnoses of GCA. As acquired images also reveal other local structures, they may assist in finding alternative diagnoses when assessing for possible GCA. We sought to assess the utility of TAMRA in identifying other significant abnormalities either associated with a diagnosis of GCA or potentially mimicking a clinical presentation of GCA. Methods A retrospective cohort study was undertaken at St Joseph's Healthcare in Hamilton, Ontario, Canada between February 2007 and April 2020 and included patients who underwent TAMRA for a possible diagnosis of GCA. Patient demographics, diagnosis and imaging findings were extracted, and descriptive analysis of findings was performed. Results We included 340 individuals who underwent TAMRA for assessment of a potential diagnosis of GCA and had clinical information available; there were 126 (37.1%) diagnoses of GCA. Fourteen (4.1%) patients had findings on TAMRA that demonstrated an alternative diagnosis, findings were predominantly in the temporomandibular joint, orbit and meninges. Eighteen (14.3%) patients with GCA had intracranial vascular changes that were demonstrative of intracranial vasculitis; one stroke was attributed to intracranial GCA. Conclusions TAMRA has proven utility in diagnosing GCA, and these data suggest that it also has utility in identifying alternative diagnoses to rule out the disease. Intracranial vasculitis was also seen in 14.3% of patients; the clinical impact of these findings is currently poorly understood and requires further study. [ABSTRACT FROM AUTHOR]

Objective To investigate the relation between biomarkers of inflammation and subsequent development of GCA. Method Participants in the population-based Malmö Diet Cancer Study (MDCS; N  = 30 447), established 1991–96, who were subsequently diagnosed with GCA, were identified in a structured process. GCA-free controls, matched for sex, year of birth and year of screening were selected from the study cohort. Baseline plasma samples were analysed using the antibody-based OLINK proteomics inflammation panel (92 inflammatory proteins). Analyses were pre-designated as hypothesis-driven or hypothesis-generating. In the latter, principal component analysis was used to identify groups of proteins that explain the variance in the proteome. Within components selected based on eigenvalues, proteins with a factor loading of >0.50 were investigated. Results Ninety-four cases with a confirmed incident diagnosis of GCA (median 11.9 years after inclusion) were identified. Among biomarkers with a priori hypotheses, IFN-γ was positively associated with GCA [odds ratio (OR) per s. d. 1.52; 95% CI 1.00, 2.30]. Eight biomarkers in the hypothesis-generating analyses were significantly associated with development of GCA. Among these, higher levels of IFN-γ (OR 2.37; 95% CI 1.14, 4.92) and monocyte chemotactic protein 3 (MCP3) (OR 4.27; 95% CI 1.26, 14.53) were particularly associated with increased risk of GCA in the subset sampled <8.5 years before diagnosis. Several other proteins known to be important for T cell function were also associated with GCA in these analyses, e.g. CXCL9, IL-2, CD40 and CCL25. Conclusion Elevated IFN-γ levels were found years prior to diagnosis of GCA. T cell activation may precede the clinical onset of GCA. [ABSTRACT FROM AUTHOR]

Objectives To compare limited with a more extended ultrasound examination (anteromedial ultrasound, A2-ultrasound) to detect large vessel (LV) involvement in patients with newly diagnosed GCA. Methods Patients with new-onset GCA were included at the time of diagnosis. All patients were examined using limited ultrasound (ultrasound of the axillary artery as visualized in the axilla) and an extended A2-ultrasound method (which also includes the carotid, vertebral, subclavian and proximal axillary arteries), in addition to temporal artery ultrasound. Results One hundred and thirty-three patients were included in the study. All patients fulfilled the criteria according to a proposed extension of the 1990 ACR classification criteria for GCA and had a positive ultrasound examination at diagnosis. Ninety-three of the 133 GCA patients (69.9%) had LV involvement when examined by extended A2-ultrasound, compared with only 56 patients (42.1%) by limited ultrasound (P  < 0.001). Twelve patients (9.0%) had vasculitis of the vertebral arteries as the only LVs involved. Five patients (3.8%) would have been missed as having GCA if only limited ultrasound was performed. Forty patients (30.0%) had isolated cranial GCA, 21 patients (15.8%) had isolated large vessel GCA and 72 patients (54.1%) had mixed-GCA. Conclusion Extended A2-ultrasound examination identified more patients with LV involvement than the limited ultrasound method. However, extended A2-ultrasound requires high expertise and high-end equipment and should be performed by ultrasonographers with adequate training. [ABSTRACT FROM AUTHOR]

We present the largest study of the frequency and nature of visual complications in a cohort of 350 patients consecutively diagnosed with giant cell arteritis (GCA). Methods All individuals were assessed using structured forms and diagnosed using imaging or biopsy. A binary logistic regression model was used to analyse data for predicting visual loss. Results Visual symptoms occurred in 101 (28.9%) patients, with visual loss in one or both eyes in 48 (13.7%) patients. Four patients had binocular visual loss. Anterior ischaemic optic neuropathy (N=31), retinal artery obstruction (N=8) and occipital stroke (N=2) were the main causes of visual loss. Of the 47 individuals who had repeat visual acuity testing at 7 days, three individuals had improvement to 6/9 or better. After introducing the fast-track pathway, the frequency of visual loss decreased from 18.7% to 11.5%. Age at diagnosis (odds ratio (OR) 1.12) and headache (OR 0.22) were significant determinants of visual loss in a multivariate model. Jaw claudication trended to significance (OR 1.96, p=0.054). Conclusions We recorded a visual loss frequency of 13.7% in the largest cohort of patients with GCA examined from a single centre. Although improvement in vision was rare, a dedicated fasttrack pathway reduced visual loss. Headache could result in earlier diagnosis and protect against visual loss. [ABSTRACT FROM AUTHOR]

Objectives To investigate the performance of cranial PET/CT for the diagnosis of GCA. Methods All patients with a suspected diagnosis of GCA were prospectively enrolled in this study and had a digital PET/CT with evaluation of cranial arteries if they had not started glucocorticoids >72 h previously. The diagnosis of GCA was retained after at least 6 months of follow-up if no other diagnosis was considered by the clinician and the patient went into remission after at least 6 consecutive months of treatment. Cranial PET/CT was considered positive if at least one arterial segment showed hypermetabolism similar to or greater than liver uptake. Results For cranial PET/CT, sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative predictive value (NPV) were 73.3%, 97.2%, 91.7% and 89.7%, respectively. For extracranial PET/CT, diagnostic performance was lower (Se = 66.7%, Sp = 80.6%, PPV = 58.8%, NPV = 85.3%). The combination of cranial and extracranial PET/CT improved overall sensitivity (Se = 80%) and NPV (NPV = 90.3%) while decreasing overall specificity (Sp = 77.8%) and PPV (PPV = 60%). Conclusion Cranial PET/CT can be easily combined with extracranial PET/CT with a limited increase in examination time. Combined cranial and extracranial PET/CT showed very high diagnostic accuracy for the diagnosis of GCA. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov , NCT05246540. [ABSTRACT FROM AUTHOR]

The purpose of this case study on giant cell arteritis (GCA) is to ensure that emergency nurse practitioners (ENPs) are knowledgeable on this disease process to prevent serious complications such as permanent blindness in patients presenting to the emergency department (ED). The case study reviews emergency management of GCA, which includes diagnostic workup, pharmacological treatment, consults, disposition, follow-up care, and when to return to the ED. The classification criteria will be discussed for GCA from the American College of Rheumatology and the European Alliance of Associations for Rheumatology. In addition, risk assessment, clinical presentation, and physical examination findings will be listed. After reviewing this case study, ENPs should be able to identify GCA and manage care to reduce complications and recurrence of GCA in a patient presenting to the ED. [ABSTRACT FROM AUTHOR]

Objective: Blindness is a feared complication of giant cell arteritis (GCA). However, the spectrum of pathologic orbital imaging findings on magnetic resonance imaging (MRI) in GCA is not well understood. In this study, we assess inflammatory changes of intraorbital structures on black blood MRI (BB-MRI) in patients with GCA compared to age-matched controls. Methods: In this multicenter case-control study, 106 subjects underwent BB-MRI. Fifty-six patients with clinically or histologically diagnosed GCA and 50 age-matched controls without clinical or laboratory evidence of vasculitis were included. All individuals were imaged on a 3-T MR scanner with a post-contrast compressed-sensing (CS) T1-weighted sampling perfection with application-optimized contrasts using different flip angle evolution (SPACE) BB-MRI sequence. Imaging results were correlated with available clinical symptoms. Results: Eighteen of 56 GCA patients (32%) showed inflammatory changes of at least one of the intraorbital structures. The most common finding was enhancement of at least one of the optic nerve sheaths (N = 13, 72%). Vessel wall enhancement of the ophthalmic artery was unilateral in 8 and bilateral in 3 patients. Enhancement of the optic nerve was observed in one patient. There was no significant correlation between imaging features of inflammation and clinically reported orbital symptoms (p = 0.10). None of the age-matched control patients showed any inflammatory changes of intraorbital structures. Conclusions: BB-MRI revealed inflammatory findings in the orbits in up to 32% of patients with GCA. Optic nerve sheath enhancement was the most common intraorbital inflammatory change on BB-MRI. MRI findings were independent of clinically reported orbital symptoms. Key Points: • Up to 32% of GCA patients shows signs of inflammation of intraorbital structures on BB-MRI. • Enhancement of the optic nerve sheath is the most common intraorbital finding in GCA patients on BB-MRI. • Features of inflammation of intraorbital structures are independent of clinically reported symptoms. [ABSTRACT FROM AUTHOR]

The article presents the case study on the effectiveness of ChatGPT versions (GPT-3.5 and GPT-4 Turbo) in providing insulin guidance for diabetes management, highlighting variations in response quality and detail. It also discusses the challenges of using AI for medical advice, the importance of accurate and clear responses for older patients, and the need for improved AI models and frameworks to ensure reliable and effective healthcare applications.

Objectives To analyse humoral and cellular immune response to mRNA COVID-19 vaccines in patients with GCA. Methods Consecutive patients with a diagnosis of GCA receiving two doses of BNT162b2 vaccine were assessed at baseline and 3 weeks from the second vaccine dose. Healthy subjects (n  = 51) were included as controls (HC). Humoral response was assessed with Spike-specific IgG antibody response (S-IgG) and neutralizing antibodies (NtAb). Specific T cell response was assessed by enzyme linked immunosorbent spot (ELISpot). Results Of 56 included patients with GCA, 44 were eligible after exclusion of previous evidence of COVID-19 and incomplete follow-up. A significant proportion of patients with GCA (91%) demonstrated antibody (S-IgG) response, but this was significantly lower than HCs (100%); P  < 0.0001. Neutralizing activity was not detected in 16% of patients with GCA. Antibody titres (S-IgG and NtAb) were significantly lower compared with HCs. Humoral response (S-IgG and NtAb) was significantly hampered by treatment with MTX. Cellular response was lacking in 30% of patients with GCA (vs 0% in HCs; P  < 0.0001). Cellular response was significantly influenced by the levels of baseline peripheral T-lymphocytes and by glucocorticoid treatment. Treatment with tocilizumab did not affect any level of the immune response elicited by vaccination. Conclusions Although patients with GCA apparently achieve a robust antibody seroconversion, there is a significant impairment of the neutralizing activity. MTX significantly reduced all levels of the humoral response. Up to one-third of patients do not develop a cellular immune protection in response to COVID-19 vaccination. [ABSTRACT FROM AUTHOR]

Background: Giant cell arteritis is an inflammatory disease of the large- and medium-sized vessels. It is the most common primary vasculitis, with lifetime incidences of 0.5% and 1% in men and women, respectively. Its diagnosis is based upon clinical criteria, which may include temporal artery biopsy. Expected positivity rates of temporal artery biopsies and patient selection remain controversial topics in the literature. Methods: A cross-sectional retrospective study of 127 patients referred for temporal artery biopsy with a diagnosis of suspected giant cell arteritis between January 2014 and December 2018 was performed. The primary outcome was the positivity rate. The relationships between positivity rates, symptoms, clinical suspicion, biopsy delay, biopsy length and corticosteroid treatment were also studied. Results: A positivity rate of 23.7% (16.6–32.6%) was shown, along with a significant association between jaw claudication and specimen positivity (odds ratio 8.1, p < 0.05). Moreover, there were significant associations between a high initial clinical suspicion of disease and specimen positivity (p < 0.05), as well as a high initial clinical suspicion of disease and pursuit of corticosteroid treatment following biopsy results, regardless of positivity (p < 0.05). The duration of corticosteroid treatment prior to biopsy was not associated with a change in positivity rate. Conclusions: The positivity rate of temporal artery biopsy was 23.7%. Treatment of patients with negative temporal artery biopsy was associated with maintenance of corticosteroid treatment when the initial clinical suspicion of arteritis was high. Therefore, temporal artery biopsy may not be necessary for patients with a high initial clinical suspicion of giant cell arteritis. [ABSTRACT FROM AUTHOR]

Background: Neuro-ophthalmologic conditions are at a higher risk of misdiagnosis compared to other ophthalmic conditions. Increased awareness of the most common diagnostic errors in neuro-ophthalmology that lead to malpractice claims can allow ophthalmologists to further improve their diagnostic workup to reduce delays in diagnosis and management, while also mitigating the risk of litigation. Methods: Malpractice trials in the Westlaw Legal Database that involved cases of neuro-ophthalmologic diagnostic errors or failures by ophthalmologists were included in this study. Results: A total of 43 cases were included, all citing failure to diagnose as the main reason for litigation. The most common diagnoses missed were cerebrovascular pathologies (30.2%), intracranial tumours (27.9%) and giant cell arteritis (25.6%). The majority of verdicts were in favour of the defendants (48.8%). After adjusting for inflation, the average amount awarded was $1,952,154. Conclusion: Nearly half of the cases resulted in a defendant verdict. Settlement and plaintiff verdicts were costly, with average awards of approximately two million inflation-adjusted dollars. Failure to diagnose cerebrovascular pathologies was the most common diagnostic error followed by failure to diagnose intracranial tumours and giant cell arteritis. It is crucial for ophthalmologists to be aware of the most common pitfalls that lead to misdiagnosis or delays in diagnosis of neuro-ophthalmologic conditions. [ABSTRACT FROM AUTHOR]

Giant cell arteritis is a rare disease that can lead to blindness in people older than 50 years. Early recognition and timely treatment can prevent irreversible damage. Early recognition can be challenging due to the variable presentation of giant cell arteritis. Patients suspected of having giant cell arteritis should be referred within 24 hours to a hospital that ideally has a fast-track clinic, including vascular imaging. Here we present two patients with giant cell arteritis that reflect a number of dilemmas regarding early recognition and treatment, the fast-track clinic and the early start of prednisolone-sparing drugs in giant cell arteritis.

Background: Giant cell arteritis (GCA) can present diagnostic challenges and early diagnosis is crucial due to potential ischemic complications. Recent guidelines suggest that a suspected diagnosis should be confirmed with temporal artery biopsy or imaging, including ultrasound (US). In our Canadian setting, point-of-care temporal artery US was near unavailable, and biopsy remains the standard of care. We hypothesize that launching a fast-track US clinic by rheumatologists may spare the need for a temporal artery biopsy. Therefore, this study aimed to assess the diagnostic performance of US in this newly launched fast-track clinic., Methods: In this single-center retrospective cross-sectional analysis, 99 visits were identified from the fast-track clinic between January 2020 and July 2022. Each subject had an US according to a standard protocol for suspicion of either new-onset or relapse of GCA. Ultrasonographers were rheumatologists who acquired training on vascular US techniques before launching the clinic. For each patient presenting with suspected new-onset GCA, the pretest probability was calculated using the Southend GCA probability score. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated using the rheumatologist clinical diagnosis as the gold standard for GCA diagnosis., Results: A total of 22 subjects had a diagnostic of GCA and 77 had another diagnostic. Patients with and without GCA were, respectively, 81.8% versus 72.7% females, had a mean age of 76.6 ± 7.7 versus. 74.8 ± 9.8 years, and a mean CRP of 73.4 ± 57.8 versus 38.3 ± 59.9 mg/l. Temporal artery US demonstrated a sensitivity of 86.3% [95% confidence interval (CI), 65.1-97.1%], a specificity of 90.9% (95% CI, 82.2-6.3%), a PPV of 73.1% (95% CI, 56.8-84.9%), and a NPV of 95.9% (95% CI, 89.0-.5%). 14 patients had a suspicion of relapse and were all correctly identified by the US. Among those with suspicion of new-onset 27, 34 and 24 US were performed for high, intermediate, and low pretest probability of GCA, respectively. The high-risk subgroup demonstrated higher PPV while similar sensitivity/specificity was observed between all three subgroups., Conclusion: Our results highlight the benefits of US as a key diagnostic tool for GCA, particularly when combined with clinical evaluations. An excellent discriminative ability for diagnosis of GCA was shown in this newly launched clinic suggesting that the role of TAB may need to be redefined. These findings will guide on broader implementation of US programs for GCA., (© The Author(s) 2024. Published by Oxford University Press on behalf of International Society for Quality in Health Care. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com.)

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

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

Importance: Prompt and accurate diagnosis of arteritic anterior ischemic optic neuropathy (AAION) from giant cell arteritis and other systemic vasculitis can contribute to preventing irreversible vision loss from these conditions. Its clinical distinction from nonarteritic anterior ischemic optic neuropathy (NAION) can be challenging, especially when systemic symptoms are lacking or laboratory markers of the disease are not reliable., Objective: To develop, train, and test a deep learning system (DLS) to discriminate AAION from NAION on color fundus images during the acute phase., Design, Setting, and Participants: This was an international study including color fundus images of 961 eyes of 802 patients with confirmed AAION and NAION. Training was performed using images from 21 expert neuro-ophthalmology centers in 16 countries, while external testing was performed in a cohort from 5 expert neuro-ophthalmology centers in the US and Europe. Data for training and external testing were collected from August 2018 to January 2023. A mix of deidentified images of 2 fields of view (optic disc centered and macula centered) were used. For training and internal validation, images were from 16 fundus camera models with fields of 30° to 55°. For external testing, images were from 5 fundus cameras with fields of 30° to 50°. Data were analyzed from January 2023 to January 2024., Main Outcomes and Measures: The performance of the DLS was measured using area under the receiver operating characteristic curve (AUC), sensitivity, specificity, and accuracy., Results: In the training and validation sets, 374 (54.9%) of patients were female, 301 (44.2%) were male, and 6 (0.9%) were of unknown sex; the median (range) age was 66 (23-96) years. When tested on the external dataset including 121 patients (35 [28.9%] female, 44 [36.4%] male, and 42 [34.7%] of unknown sex; median [range] age, 69 [37-89] years), the DLS achieved an AUC of 0.97 (95% CI, 0.95-0.99), a sensitivity of 91.1% (95% CI, 85.2-96.9), a specificity of 93.4% (95% CI, 91.1-98.2), and an accuracy of 92.6% (95% CI, 90.5-96.6). The accuracy of the 2 experts for classification of the same dataset was 74.3% (95% CI, 66.7-81.9) and 81.6% (95% CI, 74.8-88.4), respectively., Conclusions and Relevance: A DLS showing disease-specific averaged class-activation maps had greater than 90% accuracy at discriminating between acute AAION from NAION on color fundus images, at the eye level, without any clinical or biomarker information. A DLS that identifies AAION could improve clinical decision-making, potentially reducing the risk of misdiagnosis and improving patient outcomes.

Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65-0.89, p < 0.001). In those with a biopsy length of ≥ 1 cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06-1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1 cm was found to contribute towards a definitive histopathological vasculitis diagnosis., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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

An expert committee recommends defining fast-track clinics (FTC) for the acute diagnostics of giant cell arteritis (GCA) as follows: low-threshold, easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe and collaboration with partners for fast performance of neurological and ophthalmological examinations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT) and for temporal artery biopsy., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objective: To develop international consensus-based recommendations for early referral of individuals with suspected polymyalgia rheumatica (PMR)., Methods: A task force including 29 rheumatologists/internists, 4 general practitioners, 4 patients and a healthcare professional emerged from the international giant cell arteritis and PMR study group. The task force supplied clinical questions, subsequently transformed into Population, Intervention, Comparator, Outcome format. A systematic literature review was conducted followed by online meetings to formulate and vote on final recommendations. Levels of evidence (LOE) (1-5 scale) and agreement (LOA) (0-10 scale) were evaluated., Results: Two overarching principles and five recommendations were developed. LOE was 4-5 and LOA ranged between 8.5 and 9.7. The recommendations suggest that (1) each individual with suspected or recently diagnosed PMR should be considered for specialist evaluation, (2) before referring an individual with suspected PMR to specialist care, a thorough history and clinical examination should be performed and preferably complemented with urgent basic laboratory investigations, (3) individuals with suspected PMR with severe symptoms should be referred for specialist evaluation using rapid access strategies, (4) in individuals with suspected PMR who are referred via rapid access, the commencement of glucocorticoid therapy should be deferred until after specialist evaluation and (5) individuals diagnosed with PMR in specialist care with a good initial response to glucocorticoids and a low risk of glucocorticoid related adverse events can be managed in primary care., Conclusions: These are the first international recommendations for referral of individuals with suspected PMR, which complement the European Alliance of Associations for Rheumatology/American College of Rheumatology management guidelines for established PMR., Competing Interests: Competing interests: KKK: Research grants from Independent Research Fund Denmark, Danish Rheumatic Association and Central Denmark Region unrelated to this project. SES: Research grants from Rheumatology Research Foundation, Bristol Myers Squibb Foundation. Clinical trial support from AstraZeneca, GlaxoSmithKline; Consulting fees from Sanofi (funds toward research support); Data Safety Monitoring Board on MINT trial, Advisory Board for Sanofi (funds toward research support). E-MH: Has received grants unrelated to this manuscript from Novo Nordic Foundation, Roche, Novartis; Personal fees from AbbVie, Sanofi, SOBI, Merck Sharp & Dohme and Union Chimique Belge. AD: Consulting fees from Sanofi; Participation on a Data Safety Monitoring Board or Advisory Board for Sanofi; Board member Vasculitis Foundation. LN: Has received Honorarium from Abbvie; Trustee of the charity PMR-GCA Scotland. SM: Consulting fees from Astrazeneca; Honoraria from Vifor. KJW: Grants from Eli Lilly, Kiniksa, BMS; Consulting fees from Amgen, Sanofi. Honoraria from Amgen. CP: Consulting fee from Pfizer. MP: Consulting fee from Novartis; Clinical trial participant for Abbvie, Amgen, AstraZeneca. HIK: Honoraria from Roche, eTherapeutic Guidelines Australia; Board member Australian Rheumatology Association; Clinical trials for Roche, Abbvie, Sun, Emerald, Novartis, Biogen, Sanofi, Syneos. CEO: Consultancy for Abbvie; Speaking honoraria from Abbvie, Janssen, Novartis and Roche; Advisory board for Abbvie. WAS: Has received honoraria from Abbvie, Chugai, GlaxoSmithKline, Medac, Novartis, Roche, Sanofi ; Support for attending meetings/travel from Abbvie, Chugai, GlaxoSmithKline, Medac, Novartis, Roche, Sanofi; Participated in advisory board from Abbvie, GlaxoSmithKline, Novartis, Sanofi; Principle investigator of phases 2 and 3 studies sponsored by Abbvie, GlaxoSmithKlinie, Novartis and Sanofi. EBrouwer: As an employee of the UMCG received a speaker fee for a talk on GCA at a post EULAR symposium in the Netherlands in 2023 which was paid to the UMCG; As an employee of the UMCG received grants from the Dutch Arthritis Society DAS and the EU/EFPIA/Innovative Medicines Initiative 2 Joint Undertaking Immune-Image grant no 831514 which were paid to the UMCG. SM: Consultancy on behalf of her institution for Roche/Chugai, Sanofi, AbbVie, AstraZeneca, Pfizer; Investigator on clinical trials for Sanofi, GSK, Sparrow; speaking/lecturing on behalf of her institution for Roche/Chugai, Vifor, Pfizer, UCB, Novartis and AbbVie; chief investigator on STERLING-PMR trial, funded by NIHR; patron of the charity PMRGCAuk. No personal remuneration was received for any of the above activities. Support from Roche/Chugai to attend EULAR2019 in person and from Pfizer to attend ACR Convergence 2021 virtually. SM is supported in part by the NIHR Leeds Biomedical Research Centre. VD-P has received honorarium from Abbvie, Chugai, Novartis, BMS, Support for attending meetings/travel from Novartis; Participated in advisory borad from Abbvie, Novartis. All other authors have no competing interests., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ on behalf of EULAR.)

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

Background: Takayasu arteritis (TAK) and giant cell arteritis (GCA) are two major large vessel vasculitis, with varied epidemiology by geographical location, age, and race. However, the epidemiological data in Chinese population is rarely reported. This study estimated the epidemiology of TAK and GCA in Shanghainese individuals residing in China over a 10-year period., Methods: TAK data for individuals over 16 years and GCA data for individuals over 50 years were retrieved from 38 comprehensive hospitals in Shanghai, China through the electronic medical record systems between January 1, 2011, and December 31, 2020. A systematic literature review was performed to determine the global distribution of TAK and GCA by searching PubMed, Ovid-Medline, Excerpta Medica Database (EMBASE), and Web of Science., Results: In 173 identified TAK cases (67% females; mean age, 46 ± 15 years), the period prevalence was 11.72 cases per million, and the mean annual incidence was 1.33 cases per million. The highest prevalence (17.74 cases per million) and incidence (1.71 cases per million) were observed in the 16- to 34-year-old age group. In 92 identified GCA cases (56% females; age, >50 years), the period prevalence was 2.73 cases per 100 000 persons, and the mean annual incidence was 1.91 cases per 100 000 persons. Meta-analysis of the incidence study of TAK and GCA showed that the pooled incidence rate of TAK and GCA was 1.29 per million and 15.48 per 100 000 person-years, respectively. Subgroup analysis showed that the incidence of TAK was significantly higher in Asia than in other regions, while the incidence of GCA was higher in Europe, especially North Europe., Conclusion: The epidemiological patterns of TAK and GCA were comprehensively mapped globally and locally, in Shanghai, China., (© 2024 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Giant Cell Arteritis (GCA), also known as Temporal Arteritis, is a type of large vessel vasculitis primarily affecting the elderly population. It typically manifests with headaches, visual impairment, and jaw claudication. Although third nerve palsy as the primary presentation of GCA is rare, it has been reported in previous instances. In this report, we describe the case of a patient presenting with pupil-sparing third nerve palsy, ultimately diagnosed with GCA, and successfully managed with steroids and tocilizumab. A lady in her 80s with past medical history of well-controlled hypertension, bladder cancer in remission, a twenty-pack year smoking history, cervical and lumbar spine stenosis, and recent immunizations presented with acute onset of right-sided pupil-sparing third nerve palsy. Labs were pertinent for an elevated ESR and CRP. Brain imaging was without acute abnormalities. A temporal artery biopsy established evidence of healed arteritis and a diagnosis of GCA was made. The patient was treated with pulse-dose steroids followed by an oral steroid taper and tocilizumab. At one month follow-up, there was partial resolution in her ophthalmoplegia. We underscore the importance of considering temporal arteritis as a potential cause of third nerve palsy in the elderly before attributing it solely to microvascular ischemia, particularly in patients with constitutional features. Additionally, in our comprehensive literature review, we aim to consolidate the existing data from similar presentations, shedding light on the clinical manifestation and disease trajectory., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Juvenile Temporal Arteritis (JTA) is a rare non-granulomatous vasculitis affecting the superficial temporal arteries, mostly in individuals under 45 years old. It is often misdiagnosed due to its benign nature and the absence of systemic symptoms. Herein, we present a case report of a 40-year-old woman who initially presented with painless nodules in the left temporal area. Following a biopsy, the patient developed additional nodules not only in the same temple but also on the contralateral side. Remarkably, these nodules underwent spontaneous regression without further treatment, highlighting the variability in JTA's course and distinctive response to intervention. In addition, through a systematic literature review of 43 case reports - 17 with bilateral involvement - we aimed to thoroughly understand the clinical and histopathological findings, diagnostic processes, and treatment responses in JTA, with an emphasis on cases with bilateral involvement. Findings indicate that JTA typically presents as painless or painful temporal nodules, rarely accompanied by other non-specific symptoms, making histopathological examination crucial for accurate diagnosis. Collectively, our work provides the most extensive account of bilateral JTA cases to date. It emphasizes the need for clinical awareness of this condition, contributes valuable data to the limited information available on this rare condition and serves as a stepping-stone for further inquiry. The main takeaway from this review is the variable nature of JTA and the importance of histopathology in diagnosis, which helps clinicians avoid excessive testing and overtreatment and anticipate possible spontaneous resolution., (© 2024. The Author(s).)

Purpose: To describe the demographic and clinical characteristics of uveitis in patients with giant cell arteritis (GCA), their treatments, and evolution., Methods: A national retrospective cohort study was performed. The inclusion criteria were as follows: patients with GCA fulfilling the 2022 ACR/EULAR criteria and a diagnostic of uveitis attested by an ophthalmologist., Results: Seven women were included. The median age at diagnosis of uveitis was 71 years (64-84). All uveitis were diagnosed during active GCA (five at initial diagnosis, two at relapse). All uveitis were acute (100%), mostly anterior (86%) and bilateral (71%). Granulomatous features were less common (29%). All uveitis were treated with local and systemic corticosteroids. After a median follow-up of 30 (21-55) months, all patients achieved complete ophthalmic remission, with only one relapse at 2 years. GCA was also in complete remission., Conclusions: Uveitis could reveal GCA, and its presence correlated with disease activity of GCA. The most frequent clinical presentation of uveitis was acute and anterior; using local and systemic corticosteroids, the prognosis was favorable.

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.)

The article discusses a study which determined whether Giant Cell Arteritis Probability Score (GCAPS) could be applied as a risk stratification tool in both eye-specific and general cohorts and successfully predicted negative temporal artery biopsies (TAB) results in low-risk patients. Topics include the most common reported symptoms among low-risk patients and characteristics and clinical features of patients with suspected giant cell arteritis risk stratified by Southend GCAPS.

The article focuses on the complexities of managing Polymyalgia Rheumatica (PMR) and Giant Cell Arteritis (GCA) in elderly patients. Topics include the challenges of steroid tapering and relapse prediction, differences in relapse rates between PMR and GCA, and the findings from a retrospective analysis of patients diagnosed with these conditions.

Objective: Aberrant Rho-associated protein kinase (ROCK) activity is implicated in several vascular and immunologic disorders. We previously demonstrated increased ROCK activity in histopathologically negative temporal artery biopsies (TABs) in subjects with clinical giant cell arteritis (GCA) compared to those without GCA. This current study aimed to examine ROCK activity in a larger cohort of biopsy-negative GCA subjects and to validate the prior findings.Methods: Based on clinical data 6 months after TAB, subjects were categorized into 2 groups: biopsy-negative GCA and controls without GCA. Paraffin-embedded TABs were stained for phosphorylated ezrin/radixin/ moesin (pERM), a surrogate of ROCK activity, and scored by 2 pathologists blinded to clinical diagnosis using a previously derived scoring system measuring staining intensity in 3 areas of the vessel.Results: Thirty-six subjects with biopsy-negative GCA and 43 controls were analyzed. The mean (SD) pERM intensity score in non-GCA subjects was 3.9 (1.4), compared to 5.0 (1.4) in those with GCA (P = 0.002). Using the predetermined cut-off of 4 to define high pERM intensity, subjects with GCA were significantly more likely to have a high pERM intensity score compared to non-GCA (odds ratio 3.67, 95% CI 1.19-11.36; P = 0.02. The sensitivity of high pERM intensity score for diagnosis of GCA in histologically negative TABs was 86% (95% CI 70-95).Conclusion: In this well-characterized cohort, those with biopsy-negative GCA had significantly higher pERM intensity scores compared to subjects without GCA. pERM staining has diagnostic significance in enhancing the sensitivity of TAB and may help to define the clinically important group of biopsy-negative GCA. [ABSTRACT FROM AUTHOR]

Objectives GCA is a large vessel vasculitis in which metabolically active immune cells play an important role. GCA diagnosis is based on CRP/ESR and temporal artery biopsies (TABs), in combination with 18F-fluorodeoxyglucose ([18F]FDG)-PET/CT relying on enhanced glucose uptake by glycolytic macrophages. Here, we studied circulating Pyruvate Kinase M2 (PKM2), a glycolytic enzyme, as a possible systemic marker of vessel wall inflammation in GCA. Methods Immunohistochemical detection of PKM2 was performed on inflamed (n  = 12) and non-inflamed (n  = 4) TABs from GCA patients and non-GCA (n  = 9) patients. Dimeric PKM2 levels were assessed in plasma of GCA patients (n  = 44), age-matched healthy controls (n  = 41), metastatic melanoma patients (n  = 7) and infection controls (n  = 11). CRP, ESR and macrophage markers calprotectin and YKL-40 were correlated with plasma PKM2 levels. To detect the cellular source of plasma PKM2 in tissue, double IF staining was performed on inflamed GCA TABs. [18F]FDG-PET scans of 23 GCA patients were analysed and maximum standard uptake values and target to background ratios were calculated. Results PKM2 is abundantly expressed in TABs of GCA patients. Dimeric PKM2 plasma levels were elevated in GCA and correlated with CRP, ESR, calprotectin and YKL-40 levels. Elevated plasma PKM2 levels were downmodulated by glucocorticoid treatment. PKM2 was detected in both macrophages and T cells at the site of vascular inflammation. Circulating PKM2 levels correlated with average target to background ratios PET scores. Conclusion Elevated plasma PKM2 levels reflect active vessel inflammation in GCA and may assist in disease diagnosis and in disease monitoring. [ABSTRACT FROM AUTHOR]