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L'artérite à cellules géantes est une vascularite granulomateuse systémique hétérogène qui touche l'aorte et ses principales collatérales. Bien que l'atteinte des gros vaisseaux soit de plus en plus connue, les études qui décrivent l'incidence, les caractéristiques et les complications de l'artérite à cellules géantes touchant les gros vaisseaux ont des résultats contradictoires en raison d'incohérences dans les définitions de la maladie, de différences méthodologiques et de l'étendue du spectre des présentations cliniques. L'objectif de cette revue systématique de la littérature était de mieux définir l'artérite à cellules géantes avec atteinte des gros vaisseaux à partir des publications disponibles et d'identifier des caractéristiques distinctives pouvant permettre de différencier les patients atteints de cette forme d'artérite de ceux dont la maladie est limitée aux vaisseaux crâniens. Les études publiées indexées dans Medline et Embase entre la création de la base de données et le 7 mai 2021 ont été recherchées. Ont été incluses celles qui présentaient des données longitudinales ou transversales sur au moins 25 patients atteints d'artérite à cellules géantes avec atteinte des gros vaisseaux. Les groupes témoins ont été utilisés lorsqu'il existait des données sur les patients atteints d'artérite à cellules géantes limitées aux vaisseaux crâniens. Une synthèse quantitative des données a été effectuée par méta-régression avec un modèle à effets aléatoires, à l'aide du logiciel Stata. La recherche a identifié 3488 études, dont 46 ont été incluses. Les critères de diagnostic de l'artérite à cellules géantes avec atteinte des gros vaisseaux différaient entre les articles mais reposaient généralement sur l'imagerie ou l'histopathologie. Les patients présentant une atteinte des gros vaisseaux étaient globalement plus jeunes au moment du diagnostic que ceux dont la maladie était limitée aux vaisseaux crâniens (différence d'âge moyenne –4,53 ans), le délai avant leur diagnostic était plus long (différence moyenne 3,03 mois) et leurs taux de positivité de la biopsie de l'artère temporale étaient plus faibles (OR 0,52 [IC 95 % 0,3 à 0,91]). Les patients atteints d'artérite à cellules géantes avec atteinte des gros vaisseaux présentant des manifestations crâniennes étaient moins nombreux et seuls 53 % remplissaient les critères de classification de 1990 de l'ACR pour l'artérite à cellules géantes. La vascularite était détectée le plus souvent dans l'aorte thoracique, puis dans les artères sous-clavières, le tronc brachiocéphalique et les artères axillaires. La dose cumulée moyenne de prednisolone à 12 mois était de 6056,5 mg chez les patients présentant une atteinte des gros vaisseaux, les taux de récidive étaient similaires entre les deux formes et 12 % des décès de patients atteints d'artérite à cellules géantes avec atteinte des gros vaisseaux ont pu être directement attribués à une complication touchant les gros vaisseaux. Les caractéristiques de la maladie diffèrent entre les formes d'artérite à cellules géantes touchant les gros vaisseaux et uniquement les vaisseaux crâniens, ce qui a des implications sur le diagnostic, les stratégies de traitement et la surveillance des séquelles à long terme. Giant cell arteritis (GCA) is a heterogenous systemic granulomatous vasculitis involving the aorta and any of its major tributaries. Despite increased awareness of large vessel (LV) involvement, studies reporting incidence, clinical characteristics and complications of large-vessel GCA (LV-GCA) show conflicting results due to inconsistent disease definitions, differences in study methodologies and the broad spectrum of clinical presentations. The aim of this systematic literature review was to better define LV-GCA based on the available literature and identify distinguishing characteristics that may differentiate LV-GCA patients from those with limited cranial disease. Published studies indexed in MEDLINE and EMBASE were searched from database inception to 7 May 2021. Studies were included if they presented cohort or cross-sectional data on a minimum of 25 patients with LV-GCA. Control groups were included if data was available on patients with limited cranial GCA (C-GCA). Data was quantitatively synthesised with application of a random effects meta-regression model, using Stata. The search yielded 3488 studies, of which 46 were included. Diagnostic criteria for LV-GCA differed between papers, but were typically dependent on imaging or histopathology. Patients with LV-GCA were generally younger at diagnosis compared to C-GCA patients (mean age difference –4.53 years), had longer delay to diagnosis (mean difference 3.03 months) and lower rates of positive temporal artery biopsy (OR : 0.52 [95% CI : 0.3, 0.91]). Fewer LV-GCA patients presented with cranial manifestations and only 53% met the 1990 ACR Classification Criteria for GCA. Vasculitis was detected most commonly in the thoracic aorta, followed by the subclavian, brachiocephalic trunk and axillary arteries. The mean cumulative prednisolone dose at 12-months was 6056.5 mg for LV-GCA patients, relapse rates were similar between LV- and C-GCA patients, and 12% of deaths in LV-GCA patients could be directly attributed to an LV complication. Patients with LV-GCA have distinct disease features when compared to C-GCA, and this has implications on diagnosis, treatment strategies and surveillance of long-term sequalae. [ABSTRACT FROM AUTHOR]

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

Background Previous studies of mortality associated with GCA have shown conflicting results. We conducted a systematic review and meta-analysis to determine the mortality risk in GCA patients compared to the general population. Methods We searched for published studies indexed in MEDLINE and EMBASE and the Cochrane database from inception to June 18, 2015 using the terms “giant cell arteritis” and “temporal arteritis” combined with the terms for death, mortality, and survival. A manual search of citations from retrieved articles was also performed. The inclusion criteria were as follows: (1) observational studies of mortality in GCA and (2) comparison of mortality to the general population. Studies published only in abstract form were excluded. Study eligibility and quality (Newcastle–Ottawa scale) were independently assessed by at least two investigators. Random effects meta-analysis of the mortality ratio (MR) was performed by the inverse variance method. Results Out of 435 potentially relevant articles, 64 studies were reviewed, 19 studies were included in the review and 17 studies were included in the meta-analysis. Mortality was not increased in GCA patients ascertained from a population base (MR = 1.03, 95% CI: 0.96–1.10), but was increased in patients ascertained from a hospital setting (MR = 1.61, 95% CI: 1.19–2.19). There was no difference in MR by gender, and two studies provided evidence that mortality was increased in the early years following diagnosis. Conclusion At a population level, long-term mortality is not increased in GCA. However, mortality risk may be increased in some patients, and may vary over time. [ABSTRACT FROM AUTHOR]