7Imaging of vasculitis: State of the art
Introduction
Primary systemic vasculitides are classified by the diameter of the vessels that are predominantly involved. The increasing availability and improvement of imaging techniques are making a profound impact in the evaluation of patients with vasculitis, particularly for those with large vessel vasculitis (LVV), that include giant cell arteritis (GCA), Takayasu arteritis (TAK) and with primary central nervous system vasculitis (PCNSV). Available imaging techniques are ultrasound, computed tomography (CT) and computed tomography angiography (CTA), magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA), and 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET), often co-registered with computerized tomography (PET/CT) ∗[1], [2], [3]. Except for the detection of the characteristic microaneurysms alternating with stenoses in medium-sized vessel vasculitis, digital subtraction angiography (DSA) has become a therapeutic procedure for endovascular intervention in LVV rather than a diagnostic method ∗[1], [3]. Finally, in small vessel vasculitis imaging modalities are usually required to document internal organs involvement [3]. This review will focus on the role of imaging studies in diagnosing and monitoring LVV, but will also mention their principal applications in medium and small-sized vessel vasculitis. Indications and limitations of the available imaging modalities will be discussed as well.
Section snippets
Giant cell arteritis
Ultrasound depicts inflammatory artery wall thickening in LVV similar to MRI and CT. The wall thickening is most commonly concentric in axial views. It appears hypoechoic (darker) compared to the surrounding tissue. However, echogenicity is higher than the anechoic (black) artery lumen. A normal intima-media complex is a homogenous, hypo- or anechoic echostructure delineated by two parallel hyperechoic margins. In case of vasculitis thickened hypoechoic tissue with echogenicity similar to
Large vessel vasculitis
Computed tomography (CT) and CT angiography (CTA) are well suited to detect inflammatory changes in large, deep arteries because of their good spacial resolution and convenient scanning time. CT can measure aortic diameter and detect mural calcifications. CTA can evaluate both the vessel wall and the lumen of the large vessels, but cannot visualize relatively small vessels [1].
CTA has a role in diagnosing early and advanced large vessel vasculitis (LVV). In early LVV, CT may show arterial wall
Large vessel vasculitis
Similarly to CT, magnetic resonance imaging (MRI) is particularly indicated to examine the aorta and the other deep, large vessels without the use of ionizing radiation or ionidated contrast. Increased vessel wall thickness (usually with a diffuse circumferential pattern), associated with vessel wall edema on T2 and fat-suppressed sequences, and mural contrast enhancement on T1 sequences are early signs of vascular inflammation. Post-contrast T1 images are superior to T2 or fat-suppressed
Conventional angiography
Digital subtraction angiography (DSA) clearly depicts vessel luminal changes in LVV [1]. The commonest angiographic findings are long, smooth vascular stenoses, and sometimes occlusions and aneurysm [74]. Panangiography is required to determined the extent of disease involvement. However, DSA cannot demonstrate earlier vasculitic changes such as thickening of the vessel wall and mural enhancement, and is thus not useful for early diagnosis [1]. Disadvantages of DSA include its invasive nature
Positron emission tomography
18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET) is a nuclear medicine technique, currently often co-registered with computerized tomography (CT; PET/CT), which assesses the extent and amount of vascular uptake of the radiolabeled glucose analog FDG by metabolically active cells in infections, malignancies, and inflammation [1]. In active large-vessel vasculitis (LVV), there is increased FDG uptake by the vessel wall, typically with a smooth linear pattern [77]; it is unknown
Summary
Imaging studies are useful to document temporal artery inflammation in GCA and essential to show large vessel inflammation in early LVV, when vascular lesions have often not yet developed and clinical examination may thus be unrevealing. However, early imaging findings can be affected by previous immunosuppressive treatment. On the other hand, in established LVV imaging studies are useful to document the development of late vascular complications, such as stenosis, occlusion and/or dilatation.
Funding
None.
Conflict of interest statement
Wolfgang A. Schmidt: Roche (advisory board, speakers bureau), GlaxoSmithKline (advisory board, trial participation).
All the other authors declare no conflict of interest.
References (101)
- et al.
Role of imaging in the diagnosis of large and medium-sized vessel vasculitis
Rheum Dis Clin North Am
(2013) Imaging in vasculitis
Best Pract Res Clin Rheumatol
(2013)- et al.
Impact of cranial and axillary/subclavian artery involvement by color duplex sonography on response to treatment in giant cell arteritis
J Vasc Surg
(2015) - et al.
A practical guide to pediatric coronary artery imaging with echocardiography
J Am Soc Echocardiogr
(2015) - et al.
Patterns of aortic involvement in Takayasu arteritis and its clinical implications: evaluation with spiral computed tomography angiography
J Vasc Surg
(2007) - et al.
Retroperitoneal fibrosis
Lancet
(2006) - et al.
Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature
Eur J Radiol
(2005) - et al.
Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings
Chest
(2000) - et al.
Findings of cardiac magnetic resonance imaging in asymptomatic myocardial ischemic disease in Takayasu arteritis
Am J Cardiol
(2014) - et al.
Adult primary central nervous system vasculitis
Lancet
(2012)
Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update
Rheumatology (Oxford)
Imaging in systemic vasculitis
Curr Opin Rheumatol
Color duplex ultrasonography in the diagnosis of temporal arteritis
N Engl J Med
Fast track pathway reduces sight loss in giant cell arteritis: results of a longitudinal observational cohort study
Clin Exp Rheumatol
The fast-track ultrasound clinic for early diagnosis of giant cell arteritis significantly reduces permanent visual impairment: towards a more effective strategy to improve clinical outcome in giant cell arteritis?
Rheumatology (Oxford)
Ultrasound in vasculitis
Clin Exp Rheumatol
Role of ultrasound in the understanding and management of vasculitis
Ther Adv Rheum Dis
The role of ultrasound compared to biopsy of temporal arteries in the diagnosis and treatment of giant cell arteritis: a diagnostic accuracy and cost-effectiveness study
Arthritis Rheumatol
Diagnostic performance of ultrasonography for giant-cell arteritis: a meta-analysis
Ann Intern Med
Inter-Rater analysis of ultrasound and histological findings in patients with suspected giant cell arteritis
Arthritis Rheumatol
Ultrasound of proximal upper extremity arteries to increase the diagnostic yield in large-vessel giant cell arteritis
Rheumatology (Oxford)
Comparison between colour duplex sonography findings and different histological patterns of temporal artery
Rheumatology (Oxford)
Cut-off values for intima-media thickness of temporal, facial and axillary arteries in giant cell arteritis
Ann Rheum Dis
Temporal artery compression sign - a novel ultrasound finding for the diagnosis of giant cell arteritis
Ultraschall Med
The ultrasound compression sign to diagnose temporal giant cell arteritis shows an excellent interobserver agreement
Clin Exp Rheumatol
Is colour duplex sonography-guided temporal artery biopsy useful in the diagnosis of giant cell arteritis? A randomized study
Rheumatology (Oxford)
Effects of early corticosteroid treatment on magnetic resonance imaging and ultrasonography findings in giant cell arteritis
Rheumatology (Oxford)
Long-term inflammation in the temporal artery of a giant cell arteritis patient as detected by ultrasound
Ther Adv Musculoskelet Dis
Large-vessel giant cell arteritis: a cohort study
Rheumatology (Oxford)
Sonographic and clinical pattern of extracranial and cranial giant cell arteritis
Scand J Rheumatol
Diagnostic value of color Doppler ultrasonography of temporal arteries and large vessels in giant cell arteritis: a consecutive case series
Arthritis Care Res (Hoboken)
Utility of contrast-enhanced ultrasound for the assessment of the carotid artery wall in patients with Takayasu or giant cell arteritis
Eur Heart J Cardiovasc Imaging
Contrast-enhanced ultrasound of the carotid artery in patients with large vessel vasculitis: correlation with positron emission tomography findings
Arthritis Care Res (Hoboken)
2012 provisional classification criteria for polymyalgia rheumatica: a European League against Rheumatism/American College of Rheumatology collaborative initiative
Ann Rheum Dis
Performance of the new 2012 EULAR/ACR classification criteria for polymyalgia rheumatica: comparison with the previous criteria in a single-centre study
Ann Rheum Dis
Ultrasound morphological changes in the carotid wall of Takayasu's arteritis: monitor of disease progression
Int Angiol
Atherosclerotic changes in coronary aneurysms post-Kawasaki disease: in vivo demonstration with near-infrared spectroscopy and intravascular ultrasound
Eur Heart J
Polyarteritis nodosa with multiple aneurysms and renal arteriovenous fistula successfully diagnosed by colour Doppler sonography
Clin Rheumatol
Clinical and ultrasonographic evaluation of lower-extremity vein thrombosis in Behcet syndrome: an observational study
Medicine (Baltimore)
Evaluation of asymptomatic venous disease by venous Doppler ultrasonography in patients with Behcet's disease without overt thrombosis
Clin Rheumatol
Temporal arteritis: improving patient evaluation with a new protocol
Perm J
Takayasu arteritis: evaluation of the thoracic aorta with CT angiography
Radiology
Takayasu arteritis: assessment of coronary arterial abnormalities with 128-section dual-source CT angiography of the coronary arteries and aorta
Radiology
Follow-up electron beam CT for the management of early phase Takayasu arteritis
J Comput Assist Tomogr
The spectrum of findings in supra-aortic Takayasu's arteritis as seen on spiral CT angiography and digital subtraction angiography
Cardiovasc Interv Radiol
Large-vessel involvement in giant cell arteritis and polymyalgia rheumatica
Clin Exp Rheumatol
Large vessel involvement in biopsy-proven giant cell arteritis: prospective study in 40 newly diagnosed patients using CT angiography
Ann Rheum Dis
Effect of glucocorticoid treatment on computed tomography angiography detected large-vessel inflammation in giant-cell arteritis. A prospective, longitudinal study
Medicine (Baltimore)
Development of aortic aneurysm/dilatation during the followup of patients with giant cell arteritis: a cross-sectional screening of fifty-four prospectively followed patients
Arthritis Rheum
Prospective long term follow-up of a cohort of patients with giant cell arteritis screened for aortic structural damage (aneurysm or dilatation)
Ann Rheum Dis
Cited by (69)
ACR Appropriateness Criteria® Noncerebral Vasculitis
2021, Journal of the American College of RadiologyIncidence and prevalence of large vessel vasculitis (giant cell arteritis and Takayasu arteritis) in northern Italy: A population-based study
2021, Seminars in Arthritis and RheumatismCitation Excerpt :Therefore, some patients with not-evolving LV-GCA or TAK occurring before 2004 may have escaped the recording system. Furthermore, because only 65% of the patients with cranial-GCA underwent LV imaging at diagnosis, and most of them underwent only epiaortic CDS, an imaging modality with lower sensitivity for the detection of large vessel involvement compared with CTA and PET/CT, we cannot exclude an underestimation of LV-GCA [38]. Finally, our study design misses by definition prevalent cases who moved in our study population after having received the diagnosis; nevertheless, mobility is quite low in our region and the impact on prevalence should be limited.
Systemic vasculitides and the role of multitechnique imaging in the diagnosis
2021, Clinical RadiologyCitation Excerpt :Based on clinical presentation and demographics, imaging techniques can be selected. With a clinical suspicion of GCA, US/CDUS can be performed to evaluate the head and neck as well as extremity vasculature.27 As of now, the European League Against Rheumatism (EULAR) recommended vascular US/CDUS as a first-line imaging technique for patients with suspected c-GCA.8
Clinical, Laboratory and Ultrasonographic Interrelations in Giant Cell Arteritis
2021, Journal of Stroke and Cerebrovascular DiseasesImaging in large-vessel vasculitis
2020, Best Practice and Research: Clinical Rheumatology