A glossary of signs and symptoms of giant cell arteritis.

Objectives: This study seeks to create consensus-based definitions of signs and symptoms of giant cell arteritis (GCA) for use by health care professionals, primarily in research settings.

Methods: Core definitions of signs and symptoms of GCA were extracted from 11 randomised controlled trials of GCA previously reviewed in a systematic literature review conducted in the context of the development of response criteria for GCA. This information was supplemented by definitions from other sources, such as rheumatology textbooks.

Correction: Vascular ultrasound as a follow-up tool in patients with giant cell arteritis: a prospective observational cohort study.

[This corrects the article DOI: 10.3389/fmed.2024.

JAKing down glucocorticoids in giant cell arteritis.

Giant cell arteritis is a large-vessel vasculitis affecting the aorta and its branches. While treatment with glucocorticoids is efficacious in the initial management, patients experience relapses and adverse effects. The results of a clinical trial evaluating upadacitinib for treatment of giant cell arteritis have been published.

Exploring the limit of image resolution for human expert classification of vascular ultrasound images in giant cell arteritis and healthy subjects: the GCA-US-AI project.

Objectives: Prompt diagnosis of giant cell arteritis (GCA) with ultrasound is crucial for preventing severe ocular and other complications, yet expertise in ultrasound performance is scarce. The development of an artificial intelligence (AI)-based assistant that facilitates ultrasound image classification and helps to diagnose GCA early promises to close the existing gap. In the projection of the planned AI, this study investigates the minimum image resolution required for human experts to reliably classify ultrasound images of arteries commonly affected by GCA for the presence or absence of GCA.

Highlights from the breakout session: other forms of vasculitis.

The session on other forms of vasculitis included a masterful review on IgG4-related disease (IgG4-RD) following which two research studies evaluating therapeutic agents and one study on histopathologic findings of IgG4-RD were presented. Peyronel F., et al.

ANCA-associated vasculitis in patients with rheumatoid arthritis: A single-center cohort study.

Aim: To evaluate characteristics of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) in patients with rheumatoid arthritis (RA) and positive ANCA, and, to compare patients with RA+ANCA and RA+AAV.

Methods: This retrospective study included patients with RA and +ANCA. Patients with AAV were identified and data abstracted.

Management of Relapses in Giant Cell Arteritis.

Giant cell arteritis (GCA) is a relapsing large vessel vasculitis with risk of serious ischemic manifestations, including vision loss and vascular damage in the form of large artery stenosis, aneurysms, and dissections. Approximately 50% of patients treated with glucocorticoid (GC) monotherapy and 30% of patients receiving adjunctive therapy with tocilizumab experience disease relapses, often during the first 2 years after diagnosis. Although most relapses in GCA do not involve life- or organ-threatening presentations and can be controlled successfully, frequent relapses may lead to increased prescription of GC and consequent treatment-related morbidity, in addition to risk of further vascular damage.

Vascular ultrasound as a follow-up tool in patients with giant cell arteritis: a prospective observational cohort study.

Objectives: To evaluate relapses in giant cell arteritis (GCA), investigate the utility of vascular ultrasound to detect relapses, and develop and assess a composite score for GCA disease activity (GCAS) based on clinical symptoms, ultrasound imaging activity, and C-reactive protein (CRP).

Methods: Patients with GCA were prospectively followed with scheduled visits, including assessment for clinical relapse, protocol ultrasound examination, and CRP. At each visit, patients were defined as having ultrasound remission or relapse.

Relapses in giant cell arteritis: Updated review for clinical practice.

Giant cell arteritis (GCA), the most common primary vasculitis in adults, is a granulomatous systemic vasculitis usually affecting the aorta and its major branches, particularly the carotid and vertebral arteries. Although remission can be achieved in most patients with GCA using high-dose glucocorticoids (GC), relapses are frequent, occurring in >40% of GC-only treated patients, mostly during the first two years after diagnosis. Relapsing courses lead to high GC exposure, increasing the risk of treatment-related adverse effects.

Assessment of damage in Takayasu's arteritis.

Objectives: To evaluate damage and clinical characteristics associated with damage in Takayasu's arteritis (TAK).

Methods: Patients with TAK enrolled in a multicentre, prospective, observational study underwent standardized damage assessment every 6 months using the Vasculitis Damage Index (VDI) and the Large-Vessel Vasculitis Index of Damage (LVVID).

Results: The study included 236 patients with TAK: 92% female, 81% Caucasian; median (25th, 75th percentile) disease duration = 2.

Reappraisal of large artery involvement in giant cell arteritis: a population-based cohort over 70 years.

Objective: To evaluate the incidence and outcomes of large artery (LA) involvement among patients with giant cell arteritis (GCA) and to compare LA involvement to non-GCA patients.

Methods: The study included Olmsted County, Minnesota, USA residents with incident GCA between 1950 and 2016 with follow-up through 31 December 2020, death or migration. A population-based age-matched/sex-matched comparator cohort without GCA was assembled.

New Therapeutic Approaches to Large-Vessel Vasculitis.

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are large-vessel vasculitides affecting the aorta and its branches. Arterial damage from these diseases may result in ischemic complications, aneurysms, and dissections. Despite their similarities, the management of GCA and TAK differs.

Isolated Aortitis: Workup and Management.

The finding of aortitis, often incidentally noted on surgical resection, should prompt evaluation for secondary causes including large-vessel vasculitis. In a large proportion of cases, no other inflammatory cause is identified and the diagnosis of clinically isolated aortitis is made. It is unknown whether this entity represents a more localized form of large-vessel vasculitis.

Recent advances in the treatment of giant cell arteritis.

Giant cell arteritis (GCA) is a systemic, granulomatous, large-vessel vasculitis that affects individuals over the age of 50 years. Morbidity from disease includes cranial manifestations which can cause irreversible blindness, while extra-cranial manifestations can cause vascular damage with large-artery stenosis, occlusions, aortitis, aneurysms, and dissections. Glucocorticoids while efficacious are associated with significant adverse effects.

Treat-to-target recommendations in giant cell arteritis and polymyalgia rheumatica.

Objectives: To develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR).

Methods: A systematic literature review was conducted to retrieve data on treatment targets and outcomes in GCA/PMR as well as to identify the evidence for the effectiveness of a T2T-based management approach in these diseases. Based on evidence and expert opinion, the task force (29 participants from 10 countries consisting of physicians, a healthcare professional and a patient) developed recommendations, with consensus obtained through voting.

Prevalence and characteristics of giant cell arteritis in patients with newly diagnosed polymyalgia rheumatica - a prospective cohort study.

Background: It is known that giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) often occur together. So far, the prevalence of GCA in newly diagnosed PMR patients has not been evaluated in a prospective ultrasound study.

Objective: The aim of this study was to assess the prevalence of GCA using vascular ultrasound in patients with newly diagnosed PMR.

The provisional OMERACT ultrasonography score for giant cell arteritis.

Objectives: To develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties.

Methods: The OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the OMERACT ultrasonography large vessel vasculitis working group were invited to seven Delphi rounds.

Extended ultrasound examination identifies more large vessel involvement in patients with giant cell arteritis.

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.

Treatment Guidelines in Vasculitis.

The vasculitides encompass a group of inflammatory conditions affecting the blood vessels with severe consequences including tissue ischemia, structural abnormalities, such as aneurysms/dissections, and end organ damage. The different forms are commonly classified based on the size of the blood vessel involved as large-vessel, medium-vessel, and small-vessel vasculitis. The American College of Rheumatology/Vasculitis Foundation recently published guidelines on the management of several forms of primary systemic vasculitides.

Translation of cytoplasmic UBA1 contributes to VEXAS syndrome pathogenesis.

Somatic mutations in UBA1 cause vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory somatic (VEXAS) syndrome, an adult-onset inflammatory disease with an overlap of hematologic manifestations. VEXAS syndrome is characterized by a high mortality rate and significant clinical heterogeneity. We sought to determine independent predictors of survival in VEXAS and to understand the mechanistic basis for these factors.