Purpose of Review: Vasculitides are characterized by mostly autoimmunologically induced inflammatory processes of vascular structures. They have various clinical and radiologic appearances. Early diagnosis and reliable monitoring are indispensable for adequate therapy to prevent potentially serious complications. Imaging, in addition to laboratory tests and physical examination, constitutes a key component in assessing disease extent and activity. This review presents current standards and some typical findings in the context of imaging in vasculitis with particular attention to large vessel vasculitides. Recent Findings: Recently, imaging has gained importance in the management of vasculitis, especially regarding large vessel vasculitides (LVV). Recently, EULAR (European League Against Rheumatism) has launched its recommendations concerning the diagnosis of LVVs. Imaging is recommended as the preferred complement to clinical examination. Color-coded duplex sonography is considered the first choice imaging test in suspected giant cell arteritis, and magnetic resonance imaging is considered the first choice in suspected Takayasu’s arteritis. Summary: Due to diversity of clinical and radiologic presentations, diagnosis and therapy monitoring of vasculitides may constitute a challenge. As a result of ongoing technological progress, a variety of non-invasive imaging modalities now play an elemental role in the interdisciplinary management of vasculitic diseases.

Objective: Blindness is a feared complication of giant cell arteritis (GCA). However, the spectrum of pathologic orbital imaging findings on magnetic resonance imaging (MRI) in GCA is not well understood. In this study, we assess inflammatory changes of intraorbital structures on black blood MRI (BB-MRI) in patients with GCA compared to age-matched controls. Methods: In this multicenter case-control study, 106 subjects underwent BB-MRI. Fifty-six patients with clinically or histologically diagnosed GCA and 50 age-matched controls without clinical or laboratory evidence of vasculitis were included. All individuals were imaged on a 3-T MR scanner with a post-contrast compressed-sensing (CS) T1-weighted sampling perfection with application-optimized contrasts using different flip angle evolution (SPACE) BB-MRI sequence. Imaging results were correlated with available clinical symptoms. Results: Eighteen of 56 GCA patients (32%) showed inflammatory changes of at least one of the intraorbital structures. The most common finding was enhancement of at least one of the optic nerve sheaths (N = 13, 72%). Vessel wall enhancement of the ophthalmic artery was unilateral in 8 and bilateral in 3 patients. Enhancement of the optic nerve was observed in one patient. There was no significant correlation between imaging features of inflammation and clinically reported orbital symptoms (p = 0.10). None of the age-matched control patients showed any inflammatory changes of intraorbital structures. Conclusions: BB-MRI revealed inflammatory findings in the orbits in up to 32% of patients with GCA. Optic nerve sheath enhancement was the most common intraorbital inflammatory change on BB-MRI. MRI findings were independent of clinically reported orbital symptoms. Key Points: • Up to 32% of GCA patients shows signs of inflammation of intraorbital structures on BB-MRI. • Enhancement of the optic nerve sheath is the most common intraorbital finding in GCA patients on BB-MRI. • Features of inflammation of intraorbital structures are independent of clinically reported symptoms.

Giant cell arteritis (GCA) is a large vessel vasculitis, typically involving the aorta and its branches with predilection for the scalp arteries. Intracranial involvement is still part of ongoing research. We assessed inflammation of the intracranial arteries on 3D compressed sensing black-blood MRI (3D-CS-BB-MRI) in patients with GCA and age-matched controls. One hundred and five patients with 3D-CS-BB-MRI of the brain were included in this retrospective dual-centre case–control study, 55 with diagnosed GCA and 50 age-matched controls. High-resolution 3D-CS-BB-MRI was performed on a 3 T MR scanner with a post-contrast 3D-compressed-sensing MR pulse sequence, specifically a T1-weighted sampling perfection, application-optimized contrasts using different flip angle evolution (SPACE) pulse sequence with whole-brain coverage and isotropic resolution of 0.55 mm³. Two neuroradiologists blinded to clinical data independently scored the cerebral arteries qualitatively for inflammation; circumferential vessel wall thickening and contrast enhancement were scored positive for vasculitis. Eight of 55 GCA patients (14.5%) showed inflammation of at least one intracranial artery. The internal carotid artery (ICA) was affected in 6/55 (10.9%), the vertebral artery in 4/55 (7.3%) and the basilar artery and posterior cerebral artery in 1/55 (1.8%). All patients with inflammatory changes reported headaches and none showed any focal neurological deficit. Besides headache and general weakness, there was no significant correlation between inflammation of the intracranial arteries and clinical symptoms. No age-matched control patient showed inflammatory changes of the intracranial arteries. High-resolution 3D-CS-BB-MRI revealed inflammatory changes of intracranial arteries in 14.5% of GCA patients, with the intradural ICA as the most frequently affected vessel.

Our aim was to introduce a standardized system for assessing the extent of giant cell arteritis (GCA) on MRI, titled MRVAS (MR Vasculitis Activity score). To obtain a comprehensive view, we used an extensive MRI protocol including cranial vessels and the aorta with its branches. To test reliability, MRI was assessed by 4 readers with different levels of experience. 80 patients with suspected GCA underwent MRI of cranial arteries and the aorta/branches (20 vessel segments). Every vessel was rated dichotomous [inflamed (coded as 1) or not 0], providing a summed score from 0 to 20. Blinded readers (two experienced radiologists [ExR], two inexperienced radiologists [InR]) applied the MRVAS on an individual vessel and an overall level (defined as the highest score of any of the individual vessel scores). To determine interrater agreement, Cohen’s kappa was calculated for pairwise comparison of each reader for individual vessel segments. Intraclass correlation coefficients (ICC) were used for the MRVAS score. Concordance rates were excellent for both sub-cohorts on an individual vessel-based (GCA, ICC, 0.95; and non-GCA, ICC, 0.96) and Overall MRVAS score level (GCA, ICC, 0.96; and non-GCA, ICC, 1.0). Interrater agreement yielded significant concordance (p< 0.001) for all pairs (kappa range 0.78–0.98). No significant differences between ERs and IRs were observed (p= 0.38). The proposed MRVAS score allows standardized scoring of inflammation in GCA and achieved high agreement rates in a prospective setting.