巨細胞性動脈炎

OBJECTIVES: We aimed at developing French recommendations for the use of imaging modalities in Giant Cell Arteritis (GCA). METHODS: A systematic literature review was conducted to identify evidence regarding the use of Doppler ultrasound (DUS), magnetic resonance imaging (MRI), computed tomography (CT) angiography and [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET/CT) for diagnosing, monitoring, and predicting the outcome of GCA. The task force, composed of 23 physicians, proposed the recommendations through an iterative process based on evidence and expert opinion, with consensus determined by anonymous voting. RESULTS: The task force recommends an early imaging test for patients with suspected GCA. The recommendations propose DUS as the first-line imaging test for all patients with suspected GCA. FDG-PET, cranial MRI, aortic MRI and CT-scan can be used as alternative methods to assess the cranial and/or extracranial arteries, providing imaging evidence of vasculitis. Imaging of large vessels is also useful for determining prognosis and follow-up procedures. In patients with suspected visual GCA, ophtalmoscopic fundus examination, optical coherence tomography (OCT) and retinal angiography should be performed in emergency. Although imaging is not routinely recommended for follow-up, it may be used to assess a persistent vascular inflammatory process in patients suspected of relapse, especially when inflammatory biomarkers are unreliable. In this situation, vascular activity scores are recommended for follow-up. Imaging may be used to monitor long-term structural damage, particularly in areas where vascular inflammation has previously been detected. CONCLUSIONS: The recommendations offer guidance on the use of imaging for diagnosing and evaluating patients with GCA.

Aortopathies in autoimmune rheumatic diseases (ARD) include a spectrum of aortic pathologies-including aortitis, aneurysms, dissections, and insufficiency-primarily caused by systemic inflammation. This comprehensive review investigates the clinical manifestations, pathophysiology, diagnostic modalities, and management strategies across various rheumatic diseases associated with aortopathies such as large vessel vasculitis (e.g., Takayasu arteritis, giant cell arteritis), connective tissue diseases (e.g., systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis) and less common conditions (e.g., relapsing polychondritis, Cogan's syndrome, Behçet's disease, IgG4-related disease). Disease-specific pathophysiologic mechanisms of aortic wall inflammation and remodeling, including granulomatous and lymphoplasmacytic patterns and mixed inflammatory infiltrates, are described. Diagnostic imaging modalities-such as CTA, MRI, and PET/CT-are evaluated for their roles in detecting active inflammation, assessing structural complications, and guiding clinical decision-making. Histopathological findings provide insight into disease-specific vascular changes. Management strategies focus on the use of glucocorticoids, disease-modifying antirheumatic drugs (DMARDs), and biologics, including IL-6 and TNF-α inhibitors, with an emphasis on patient-centered approaches, multidisciplinary care, and timely surgical intervention for complications. Evidence gaps include optimal screening intervals and the role of novel biomarkers in risk stratification and in monitoring disease progression, highlighting the need for early recognition, frequent monitoring, and aggressive management of aortic involvement in rheumatic diseases to prevent life-threatening complications.

Medically complex presentations may pose a diagnostic challenge for providers, highlighting the need for inter-team communication and independent imaging review. We report a single case in which the patient presented with cranial nerve III palsy from a posterior communicating artery (PCOM) aneurysm that was missed on three separate angiographies. The patient initially presented with an incomplete cranial nerve (CN) III palsy and had workup and treatment for giant cell arteritis (GCA). Three months later, she presented again with worsened, complete CN III palsy. Upon independent review of CT and MRI angiography by the ophthalmology and neuro-radiology team, an 8 mm aneurysm of the right internal carotid artery (ICA) adjacent to the posterior communicating artery (PCOM) (initially missed) was identified. The patient was treated with percutaneous coil embolization, which resulted in partial recovery of her CN III function. This case demonstrates the importance of maintaining a high index of suspicion, independently reviewing radiologic imaging, and inter-team discussion.

A 75-year-old woman presented with a sudden onset of left homonymous hemianopia and dizziness without headache. Magnetic resonance imaging revealed multiple posterior circulation infarctions, and magnetic resonance angiography revealed right vertebral artery occlusion and left vertebral artery stenosis. Vessel wall imaging (VWI) revealed concentric wall thickening with enhancement, suggesting vasculitis. 18F-FDG PET/CT revealed an increased aortic uptake. Despite elevated CRP and ESR levels, the temporal artery ultrasound was normal. A biopsy confirmed giant cell arteritis (GCA). Prednisolone and tocilizumab normalized the inflammatory markers. This case shows that GCA can present without classic symptoms, and VWI may help evaluate unexplained vascular lesions without conventional risk factors.

Anterior ischemic optic neuropathy (AION) is one of the leading causes of sudden, painless, monocular vision loss in individuals over 50 years of age. Two forms can be distinguished, with clearly different clinical, diagnostic, and therapeutic implications: the arteritic form, most commonly associated with giant cell arteritis (GCA), which constitutes a medical and ophthalmological emergency; and the non-arteritic form, which is far more frequent and for which no specific effective treatment is currently available. Despite its clinical relevance and the frequent coexistence of vascular risk factors, the management of AION remains heterogeneous, with considerable variability in care pathways, etiological assessment, and follow-up and secondary prevention strategies. This situation highlights the need for a comprehensive and coordinated approach, in which close collaboration between Ophthalmology and Internal Medicine is essential. This position statement is part of a series of joint working groups documents promoted by the Vascular Risk Group of the Spanish Society of Internal Medicine (SEMI) and the Spanish Retina and Vitreous Society (SERV), which began with the consensuses on retinal arterial and venous occlusion. Internists with extensive experience in vasculitis and systemic diseases, who are members of the Systemic Autoimmune Diseases Group (GEAS), have participated in the preparation of this text. The aim is to establish a consensus-based proposal for the clinical management of AION. The epidemiological, pathophysiological, diagnostic and therapeutic aspects of the disease are critically reviewed, and practical recommendations are provided to improve early detection, etiological management and the prevention of visual and systemic complications, with particular emphasis on the role of the internist in the care of these patients. The recommendations included in this document should be interpreted as expert consensus-based recommendations, particularly in those sections where high‑quality evidence is lacking.