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Over the last three years, the Coronavirus-19 disease has been a global health emergency, playing a primary role in the international scientific community. Clinical activity and scientific research have concentrated their efforts on facing the pandemic, allowing the description of novel pathologies correlated to Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), such as the Multisystemic Inflammatory Syndrome in Children and Adults (MIS-C, MIS-A). Conversely, this shift of attention to COVID-19 disease and its complications could, in some cases, have delayed and underestimated the diagnosis of diseases not associated with SARS-CoV-2, including rare diseases. Here we describe the diagnostic process that led to the definition of a rare vasculitis in a young woman with a recent clinical history of SARS-CoV-2. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are the major forms of large vessel vasculitis (LVV). Glucocorticoids represent the cornerstone of LVV treatment, however, relapses and recurrences frequently occur when they are tapered or stopped, determining a prolonged exposure to glucocorticoids and a subsequent increased risk of glucocorticoid-related side effects. Therefore, conventional and biologic immunosuppressive drugs have been proposed to obtain a glucocorticoid-sparing effect. We searched PubMed® using the keywords 'giant cell arteritis/drug therapy' and 'Takayasu Arteritis/drug therapy' OR 'Takayasu Arteritis/surgery' This review focuses on the management of LVV, based on the current evidence while highlighting the differences in terms of therapeutic management of TAK and GCA. Conventional disease modifying anti-rheumatic drugs, such as methotrexate or azathioprine, are recommended in association to glucocorticoids for selected GCA and all TAK patients. Two randomized placebo-controlled trials recently demonstrated the efficacy of tocilizumab in reducing relapses and cumulative prednisone dosage in GCA patients with newly diagnosed or relapsing disease. Observational evidence and two small randomized controlled trials support the use of TNF-alpha inhibitors and tocilizumab as glucocorticoid-sparing agents in relapsing TAK, albeit high-quality evidence regarding the management of TAK is still lacking. [ABSTRACT FROM AUTHOR]

Over the last three years, the Coronavirus-19 disease has been a global health emergency, playing a primary role in the international scientific community. Clinical activity and scientific research have concentrated their efforts on facing the pandemic, allowing the description of novel pathologies correlated to Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), such as the Multisystemic Inflammatory Syndrome in Children and Adults (MIS-C, MIS-A). Conversely, this shift of attention to COVID-19 disease and its complications could, in some cases, have delayed and underestimated the diagnosis of diseases not associated with SARS-CoV-2, including rare diseases. Here we describe the diagnostic process that led to the definition of a rare vasculitis in a young woman with a recent clinical history of SARS-CoV-2.

Giant cell arteritis (GCA) is a large-vessel granulomatous vasculitis occurring in patients over 50-year-old. Diagnosis can be challenging because there is no specific biological test or other diagnoses to consider. Two main phenotypes of GCA are distinguished and can be associated. First, cranial GCA, whose diagnosis is usually confirmed by the evidence of a non-necrotizing granulomatous panarteritis on temporal artery biopsy. Second, large-vessel GCA, whose related symptoms are less specific (fever, asthenia, and weight loss) and for which other diagnoses must be implemented if there is neither cephalic GCA nor associated polymyalgia rheumatica (PMR) features chronic infection (tuberculosis, Coxiella burnetti), IgG4-related disease, Erdheim Chester disease, and other primary vasculitis (Behçet disease, relapsing polychondritis, or VEXAS syndrome). Herein, we propose a review of the main differential diagnoses to be considered regarding large vessel vasculitis.

Résumé L’imagerie est indispensable pour le diagnostic de l’aortite, l’évaluation du caractère inflammatoire des lésions artérielles, l’évaluation de la réponse thérapeutique et le dépistage des complications vasculaires à long terme. Deux situations peuvent conduire au diagnostic d’aortite au cours de l’artérite à cellules géantes (ACG) : des symptômes généraux non spécifiques ou la recherche d’une aortite au cours d’une ACG confirmée. Le taux de détection de l’aortite est variable selon la méthode d’imagerie utilisée : environ 40 % des patients avec l’imagerie conventionnelle (scanner et IRM), et environ 60 % en TEP au FDG. L’impact clinique et pronostique de la détection systématique de l’aortite au cours de l’ACG reste à déterminer. Dans la maladie de Takayasu, les indications de l’imagerie sont le diagnostic de l’atteinte artérielle, le bilan d’extension des lésions et l’évaluation de l’activité inflammatoire des lésions artérielles. Les critères d’imagerie pour évoquer une inflammation vasculaire sont la modification de l’épaisseur pariétale, l’intensité du rehaussement, la présence d’un œdème pariétal et une fixation de FDG (> foie). Cependant, la valeur de ces critères, ainsi que leur seuil de positivité sont mal connus. Ainsi, la valeur de ces examens, en particulier la TEP-FDG et l’IRM, et des différents paramètres utilisés pour la caractérisation de l’inflammation vasculaire infra-clinique reste encore à déterminer. The imaging is essential for the diagnosis of large vessels arteritis, in order to assess the persistent inflammation of arterial lesions, to evaluate the treatment response and search the vascular complications. In patients with giant cell arteritis (GCA), the aortitis could be suspected in 2 situations: in the presence of general constitutional symptoms or systematic screening of aortitis in patient with confirmed GCA. The frequency of aortitis varies according to the imaging method and could be detected in 40 % of patients with computed tomography and MRI, and approximately in 60 % with FDG-PET/CT. The clinical and prognostic value of systematic detection of aortitis during the GCA remains to be determined. In Takayasu arteritis, imaging is performed to diagnose the large vessels vasculitis, to determine the arterial lesions extension to assess the persistent inflammation of arterial lesions. The persistent vascular inflammation should be suspected in the presence of arterial thickness, of arterial enhancement, a parietal edema and increased arterial FDG uptake (> liver). However, the value of these parameters and the threshold remain to be determined. Thus, the value of FDG-PET/CT and MRI and of parameters used to characterize the persistent arterial inflammation should be further studied. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) is a large-vessel granulomatous vasculitis occurring in patients over 50-year-old. Diagnosis can be challenging because there is no specific biological test or other diagnoses to consider. Two main phenotypes of GCA are distinguished and can be associated. First, cranial GCA, whose diagnosis is usually confirmed by the evidence of a non-necrotizing granulomatous panarteritis on temporal artery biopsy. Second, large-vessel GCA, whose related symptoms are less specific (fever, asthenia, and weight loss) and for which other diagnoses must be implemented if there is neither cephalic GCA nor associated polymyalgia rheumatica (PMR) features chronic infection (tuberculosis, Coxiella burnetti), IgG4-related disease, Erdheim Chester disease, and other primary vasculitis (Behçet disease, relapsing polychondritis, or VEXAS syndrome). Herein, we propose a review of the main differential diagnoses to be considered regarding large vessel vasculitis. [ABSTRACT FROM AUTHOR]

The imaging is essential for the diagnosis of large vessels arteritis, in order to assess the persistent inflammation of arterial lesions, to evaluate the treatment response and search the vascular complications. In patients with giant cell arteritis (GCA), the aortitis could be suspected in 2 situations: in the presence of general constitutional symptoms or systematic screening of aortitis in patient with confirmed GCA. The frequency of aortitis varies according to the imaging method and could be detected in 40 % of patients with computed tomography and MRI, and approximately in 60 % with FDG-PET/CT. The clinical and prognostic value of systematic detection of aortitis during the GCA remains to be determined. In Takayasu arteritis, imaging is performed to diagnose the large vessels vasculitis, to determine the arterial lesions extension to assess the persistent inflammation of arterial lesions. The persistent vascular inflammation should be suspected in the presence of arterial thickness, of arterial enhancement, a parietal edema and increased arterial FDG uptake (>liver). However, the value of these parameters and the threshold remain to be determined. Thus, the value of FDG-PET/CT and MRI and of parameters used to characterize the persistent arterial inflammation should be further studied., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)