Search Results (22)

Background and Clinical Significance: Giant cell arteritis (GCA) is an autoimmune vasculitis of both medium and large-sized vessels typically affecting females 50 years of age or older. Severe complications can include permanent visual loss, acute coronary syndrome, or stroke. This case will present an atypical presentation of bilateral OPN which can be a rare manifestation of GCA; Case Report: Our patient developed acute, painful worsening central vision loss progressing from right eye to left with bilateral extraocular motility restriction and magnetic resonance image (MRI) revealed bilateral, circumferential optic nerve sheath enhancement suggesting optic perineuritis (OPN). Temporal artery biopsy confirmed GCA with bilateral temporal arteritis. The patient was treated with a high dose course of corticosteroids followed by a taper and was started on upadacitinib with symptomatic improvement; Conclusions: This case underscores OPN as a rarer manifestation of giant cell arteritis that can present with bilateral painful eye movements and vision loss. Early recognition and prompt corticosteroid therapy are essential to prevent irreversible visual impairment. Full article

Giant cell arteritis (GCA) represents one of the most diagnostically challenging systemic vasculitides, characterized by its heterogeneous clinical presentation, lack of pathognomonic features, and potential for devastating complications, with a special concern for irreversible vision loss. This comprehensive review synthesizes current evidence regarding the multifaceted diagnostic challenges in GCA, incorporating recent advances in classification criteria, imaging technologies, biomarker research, and emerging therapeutic strategies. Full article

Background: Giant cell arteritis (GCA) is a systemic granulomatous vasculitis affecting large and medium-sized arteries, predominantly in individuals over 50 years. While it traditionally involves cranial branches of the external carotid artery, particularly the temporal arteries, growing evidence underscores frequent extracranial involvement, especially in the supra-aortic trunks. Objective: We aimed to critically review the diagnostic utility of extended Color Doppler Ultrasound (CDUS) in GCA, with a focus on vertebrobasilar involvement and current international imaging guidelines. Methods: Taking inspiration from a representative case of extracranial GCA with vertebrobasilar ischemic events, the current literature and international recommendations (e.g., EULAR, ACR, BSR and SIR) were reviewed. Results: Diagnostic accuracy significantly improves when CDUS is extended to include carotid, vertebral, subclavian and axillary arteries. Elevated inflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) correlate with large-vessel involvement and support the use of extended scanning protocols. International guidelines vary in their emphasis on extended CDUS, but consensus is growing toward ultrasound imaging-first strategies in expert hands. Conclusion: Extended CDUS is a sensitive, non-invasive first-line diagnostic tool for GCA. In patients with symptoms of the posterior cerebral circulation and elevated inflammatory indices, early comprehensive vascular imaging reduces diagnostic delay and may obviate the need for temporal artery biopsy. Full article

Background: Early diagnosis of giant cell arteritis (GCA) is crucial to avoid loss of vision, but detailed headache characteristics of GCA have been poorly studied. Clinical prediction rules have shown promise in guiding management decisions in suspected GCA. Methods: This is a prospective, monocentric cohort study on patients ≥50 years of age with suspected GCA. The diagnostic efficacy and safety of a previously published prediction rule embedded in a stepwise diagnostic algorithm is compared to the final clinical diagnosis incorporating the results of temporal artery biopsy (TAB). The protocol of the ongoing study is presented in detail. Based on an interim analysis of the first 50 included patients, characteristics of cranial symptoms of patients with positive and negative TAB are compared, and a modification of the original prediction rule is presented. Results: TAB was positive in 23 and negative in 26 cases. In one patient, the TAB specimen contained no arterial segment, so this patient was excluded from the interim analysis. Headache was more commonly located temporally and bilaterally. Cranial ischemic symptoms and superficial temporal artery-related symptoms were more common in patients with positive TAB. The quality and intensity of headaches did not differ significantly between groups. As the original prediction rule misclassified a single patient who eventually had a positive TAB, the clinical prediction rule was modified. Conclusions: Given the limited sensitivity and specificity of cranial symptoms, a stepwise diagnostic algorithm based on the modified prediction rule may facilitate clinical decision-making in suspected GCA. Full article

Giant cell arteritis (GCA) is a rare disease of the arteries, occurring mainly in the elderly. Although the involvement of temporal arteries can be mostly symptomatic, the occlusion of ophthalmic arteries has severe consequences. GCA affecting temporal arteries is an emergency requiring quick commencement of treatment with glucocorticoids due to the serious consequences of neglect—blindness. According to the new guidelines released by EULAR, ultrasound is the tool of choice in support of the clinical diagnosis of giant cell arteritis, replacing temporal artery biopsy (TAB), as it is a sensitive and non-invasive method that is widely available. The main limitation is that the reliability of this imaging is based on the technical expertise of ultrasonographers. However, performing imaging should not delay commencing the treatment. In this work, we present ultrasound images from a case report of a 74-year-old female patient where difficulties in establishing a diagnosis led to vision loss in both eyes. In this example, we describe the ultrasound findings in giant cell arteritis, emphasizing its usefulness in supporting a diagnosis of GCA. Full article

In order to support or refute the clinical suspicion of cranial giant cell arteritis (GCA), a supplemental imaging modality is often required. High-resolution black blood Magnetic Resonance Imaging (BB MRI) techniques with contrast enhancement can visualize artery wall inflammation in GCA. We compared findings on BB MRI without contrast enhancement with findings on 2-deoxy-2-[¹⁸F]fluoro-D-glucose positron emission tomography/low-dose computed tomography (2-[¹⁸F]FDG PET/CT) in ten patients suspected of having GCA and in five control subjects who had a 2-[¹⁸F]FDG PET/CT performed as a routine control for malignant melanoma. BB MRI was consistent with 2-[¹⁸F]FDG PET/CT in 10 out of 10 cases in the group with suspected GCA. In four out of five cases in the control group, the BB MRI was consistent with 2-[¹⁸F]FDG PET/CT. In this small population, BB MRI without contrast enhancement shows promising performance in the diagnosis of GCA, and might be an applicable imaging modality in patients. Full article

Giant cell arteritis (GCA) is a chronic vasculitis that primarily affects the elderly, and can cause visual impairment, requiring prompt diagnosis and treatment. The global impact of the coronavirus disease 2019 (COVID-19) pandemic has been substantial. Although vaccination programs have been a key defense strategy, concerns have arisen regarding post-vaccination immune-mediated disorders and related risks. We present a case of GCA after COVID-19 vaccination with 2 years of follow-up. A 69-year-old woman experienced fever, headaches, and local muscle pain two days after receiving the COVID-19 vaccine. Elevated inflammatory markers were observed, and positron emission tomography (PET) revealed abnormal uptake in the major arteries, including the aorta and subclavian and iliac arteries. Temporal artery biopsy confirmed the diagnosis of GCA. Treatment consisted of pulse therapy with methylprednisolone, followed by prednisolone (PSL) and tocilizumab. Immediately after the initiation of treatment, the fever and headaches disappeared, and the inflammation markers normalized. The PSL dosage was gradually reduced, and one year later, a PET scan showed that the inflammation had resolved. After two years, the PSL dosage was reduced to 3 mg. Fourteen reported cases of GCA after COVID-19 vaccination was reviewed to reveal a diverse clinical picture and treatment response. The time from onset of symptoms to GCA diagnosis varied from two weeks to four months, highlighting the challenge of early detection. The effectiveness of treatment varied, but was generally effective similarly to that of conventional GCA. This report emphasizes the need for clinical vigilance and encourages further data collection in post-vaccination GCA cases. Full article

Dynamic contrast-enhanced MRI (DCE) is an emerging modality in the study of vertebral body malignancies. DCE-MRI analysis relies on a pharmacokinetic model, which assumes that contrast uptake is simultaneous in the feeding of arteries and tissues of interest. While true in the highly vascularized brain, the perfusion of the spine is delayed. This delay of contrast reaching vertebral body lesions can affect DCE-MRI analyses, leading to misdiagnosis for the presence of active malignancy in the bone marrow. To overcome the limitation of delayed contrast arrival to vertebral body lesions, we shifted the arterial input function (AIF) curve over a series of phases and recalculated the plasma volume values (V_p) for each phase shift. We hypothesized that shifting the AIF tracer curve would better reflect actual contrast perfusion, thereby improving the accuracy of V_p maps in metastases. We evaluated 18 biopsy-proven vertebral body metastases in which standard DCE-MRI analysis failed to demonstrate the expected increase in V_p. We manually delayed the AIF curve for multiple phases, defined as the scan-specific phase temporal resolution, and analyzed DCE-MRI parameters with the new AIF curves. All patients were found to require at least one phase-shift delay in the calculated AIF to better visualize metastatic spinal lesions and improve quantitation of V_p. Average normalized V_p values were 1.78 ± 1.88 for zero phase shifts (P0), 4.72 ± 4.31 for one phase shift (P1), and 5.59 ± 4.41 for two phase shifts (P2). Mann–Whitney U tests obtained p-values = 0.003 between P0 and P1, and 0.0004 between P0 and P2. This study demonstrates that image processing analysis for DCE-MRI in patients with spinal metastases requires a careful review of signal intensity curve, as well as a possible adjustment of the phase of aortic AIF to increase the accuracy of V_p. Full article

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is reported to induce and augment autoimmune processes. Moreover, postinfectious effects of coronavirus disease 2019 (COVID-19) are still poorly understood and often resemble symptoms of the acute infection phase. A patient with swollen extremities was presented to the Department of Angiology at the Medical University of Vienna with complaints of muscle and joint pain, paresthesia, and arterial hypertension with intense headache. Prior to these complaints, she had been suffering from various symptoms since November 2020, following a SARS-CoV-2 infection in the same month. These included recurrent sore throat, heartburn, dizziness, and headache. Paresthesia and muscle and joint pain started in temporal relation to a human papillomavirus (HPV) vaccination. Since the patient was suffering from severe pain, intensive pain management was performed. Skin and nerve biopsies revealed autoimmune small fiber neuropathy. The patient’s condition could be related to COVID-19, as her first symptoms began in temporal relation to the SARS-CoV-2 infection. Furthermore, in the disease course, antinuclear (ANA) and anti-Ro antibodies, as well as anti-cyclic citrullinated peptide (anti-CCP) antibodies, could be detected. Together with the symptoms of xerophthalmia and pharyngeal dryness, primary Sjögren’s syndrome was diagnosed. In conclusion, though biopsy results could not distinguish a cause of the disease, SARS-CoV-2 infection can be discussed as a likely trigger for the patient’s autoimmune reactions. Full article

Giant cell arteritis (GCA) is an ophthalmological emergency that can be difficult to diagnose and prompt treatment is vital. We investigated the sequential diagnostic value for patients with suspected GCA using three biochemical measures as they arrive to the clinician: first, platelet count, then C-reactive protein (CRP), and lastly, erythrocyte sedimentation rate (ESR). This retrospective cross-sectional study of consecutive patients with suspected GCA investigated platelet count, CRP, and ESR using diagnostic test accuracy statistics and odds ratios (ORs) in a sequential fashion. The diagnosis was established by experts at follow-up, considering clinical findings and tests including temporal artery biopsy. A total of 94 patients were included, of which 37 (40%) were diagnosed with GCA. Compared with those without GCA, patients with GCA had a higher platelet count (p < 0.001), CRP (p < 0.001), and ESR (p < 0.001). Platelet count demonstrated a low sensitivity (38%) and high specificity (88%); CRP, a high sensitivity (86%) and low specificity (56%); routine ESR, a high sensitivity (89%) and low specificity (47%); and age-adjusted ESR, a moderate sensitivity (65%) and moderate specificity (65%). Sequential analysis revealed that ESR did not provide additional value in evaluating risk of GCA. Initial biochemical evaluation can be based on platelet count and CRP, without waiting for ESR, which allows faster initial decision-making in GCA. Full article

We report a 78-year-old man presenting with persistent headaches in vertex and temporo-parietal area; fatigue, worsening after walking; jaw claudication; scotomas; pharyngodynia; and dry cough after the second dose of the SARS-CoV-2 vaccine (ChAdOx1-S) administration. Laboratory findings showed an elevated C-reactive protein level and FDG-CT PET showed evidence of active large vessel vasculitis with diffuse abnormal artery uptake. Under suspicion of vasculitis, a temporal arteries biopsy was performed; the histopathologic findings demonstrated the transmural inflammatory infiltrate with giant cells, compatible with giant cell arteritis. Although the overall incidence of vaccine-triggered autoimmunity is low, rheumatologists worldwide should be aware of autoimmune diseases as a new potential adverse event of vaccines. Full article

The aim of this study was to assess the interrelation between vascular ultrasonography (US) findings, histopathological data, and the expression of selected dysregulated microRNAs (miRNAs) in giant cell arteritis (GCA). The study included data on the clinical parameters, US measurements, and temporal artery biopsies (TABs) of 46 treatment-naïve patients diagnosed with GCA and 22 age-matched non-GCA patient controls. We performed a comprehensive comparative and correlation analysis along with generation of receiver operating characteristic (ROC) curves to ascertain the diagnostic performance of US examination parameters and selected miRNAs for GCA diagnosis. We showed significant differences in the US-measured intima–media thickness of the temporal arteries, the presence of a halo sign, and the presence of luminal stenosis between GCA-positive/TAB-positive, GCA-positive/TAB-negative, and non-GCA patients. Correlation analysis revealed significant associations between several histopathological parameters, US-measured intima–media thickness, and the halo sign. We found that the significant overexpression of miR-146b-5p, miR-155-5p, miR-511-5p, and miR-21-5p, and the under-expression of the miR-143/145 cluster, miR-30a-5p, and miR-125a-5p, coincides and is associated with the presence of a halo sign in patients with GCA. Notably, we determined a high diagnostic performance of miR-146b-5p, miR-21-3p, and miR-21-5p expression profiles in discriminating GCA patients from non-GCA controls, suggesting their potential utilization as putative biomarkers of GCA. Taken together, our study provides an insight into the US-based diagnostic evaluation of GCA by revealing the complex interrelation of clearly defined image findings with underlying vascular immunopathology and altered arterial tissue-specific miRNA profiles. Full article

Polymyalgia rheumatica (PMR) is an inflammatory rheumatism of the shoulder and pelvic girdles. In 16 to 21% of cases, PMR is associated with giant cell arteritis (GCA) that can lead to severe vascular complications. Ruling out GCA in patients with PMR is currently a critical challenge for clinicians. Two GCA phenotypes can be distinguished: cranial GCA (C-GCA) and large vessel GCA (LV-GCA). C-GCA is usually suspected when cranial manifestations (temporal headaches, jaw claudication, scalp tenderness, or visual disturbances) occur. Isolated LV-GCA is more difficult to diagnose, due to the lack of specificity of clinical features which can be limited to constitutional symptoms and/or unexplained fever. Furthermore, many studies have demonstrated the existence—in varying proportions—of subclinical GCA in patients with apparently isolated PMR features. In PMR patients, the occurrence of clinical features of C-GCA (new onset temporal headaches, jaw claudication, or abnormality of temporal arteries) are highly predictive of C-GCA. Additionally, glucocorticoids’ resistance occurring during follow-up of PMR patients, the occurrence of constitutional symptoms, or acute phase reactants elevation are suggestive of associated GCA. Research into the predictive biomarkers of GCA in PMR patients is critical for selecting PMR patients for whom imaging and/or temporal artery biopsy is necessary. To date, Angiopoietin-2 and MMP-3 are powerful for predicting GCA in PMR patients, but these results need to be confirmed in further cohorts. In this review, we discuss the diagnostic challenges of subclinical GCA in PMR patients and will review the predictive factors of GCA in PMR patients. Full article

Introduction: The American College of Rheumatology (ACR) criteria, and more recently the revised ACR criteria (rACR), are a scoring system developed to aid in the diagnosis of giant cell arteritis (GCA). Our aim was to investigate the value of the non-biopsy criteria of the original ACR criteria and rACR criteria to predict GCA, and investigate the utilization of such scores to avoid biopsy when a very high or very low likelihood of a positive temporal artery biopsy TAB was predicted. Method: We conducted a retrospective cohort study of 59 patients undergoing TAB from 2013 to 2017 in Beaumont Hospital, a tertiary referral centre in Dublin, Ireland. Demographic data, biochemical results, presenting features, and histology results were collected and collated. Results: Data were analysed from 53 patients and ACR scores were compiled. Seventeen scored < 3 and thirty-six scored 3–5. All 11 positive biopsies were in the 3–5 score range. Forty-five patients were analysed with rACR scores. Eight were excluded due to not meeting the inclusion criteria. Of the 11 positive biopsies, 2 were in the 3–4 score range, and 9 were in the ≥5 score range. In the ACR method, 36% of all biopsies scored as low-risk pre-biopsy. In the rACR method, 84.4% of all biopsies scored in the low- and intermediate-risk group pre-biopsy and 15.6% of all biopsies scored in the high-risk group pre-biopsy. Conclusions: This study illustrates the potential value of the rACR scoring system as a useful tool to categorize patients according to risk with a view to avoiding unnecessary TAB. The data suggest that a TAB has a helpful role in low- and intermediate-risk groups but is of minimal benefit in the high-risk group. Full article

Background: The assessment of giant cell arteritis (GCA) in fast-track assessment clinics (FTA) including the use of ultrasound (US) is becoming the preferred practice in specialized centers. Methods: Patients with suspected GCA referred to the FTA of the Rheumatology Department, University of Pavia, Italy, between 2016 and 2021 were included to analyze the clinical and US findings. Results: A total of 553 US examinations were performed on 347 patients. A total of 246 were female (71%), and the mean age was 73 ± 12. Of these, 287 US on newly referred patients led to a confirmed diagnosis of GCA in 111 (39%). The sensitivity of US was 81.98% (95% CI 73.55–88.63%), and the specificity 99.43% (95% CI 96.88–99.99%). Only 4 patients required temporal artery biopsy. The most specific symptoms to inform the pre-test probability of GCA and differentiate from patients with other conditions were: jaw or tongue claudication, scalp tenderness, and bilateral visual loss. Headache was not reported in 33% of patients. Systemic symptoms were significantly more frequent in GCA (42.3%), together with combinations of cranial, systemic, and/or polymyalgia rheumatica symptoms. Out of 88 patients, there were 52% with a confirmed relapse. Of these, 67% had a positive US. Conclusion: The use of FTA in clinical practice ensures an early diagnosis, avoiding invasive procedures for the patient. Our data support the increasingly recognized adjunctive role of US in the monitoring of GCA. Full article