Idiopathic Aortitis Research Articles

P176 A retrospective analysis of aortitis cases from radiology reports in a London teaching hospital: implications for screening and management

Abstract Background Aortitis is a heterogeneous rare condition causing aortic inflammation, often due to infectious or autoimmune aetiology. Its presentation, diagnosis and optimal management remain poorly understood, often requiring multidisciplinary input. With the advent of improved imaging techniques, an increasing number of radiology reports are identifying features of vasculitis necessitating clinical investigation. We investigated how cases identified on imaging with features of aortitis reflected clinical diagnosis and treatment. We further examined whether radiological detection of aortitis enables more rapid management decisions to improve outcomes. Methods A search was conducted of all radiology reports from St George’s Hospital between 2008 and 2018 for key words indicating “aortitis”, including ‘hyperintense vessel uptake’ and ‘periaortic inflammation.’ From 15,963 initial results, 80 reports were identified with radiological suspicion of aortitis. Results Of 80 cases, 36/80 were diagnosed with autoimmune or idiopathic inflammatory pathology: 9 retroperitoneal fibrosis, 8 idiopathic, 7 Takayasu’s, 4 giant cell arteritis, 2 inflammatory aneurysms, 2 Behçet's, 2 IgG4 disease, 1 lupus, 1 granulomatosis with polyangiitis. Diagnosis of autoimmune or idiopathic aortitis was based on symptomatology, imaging, serology, vessel biopsy, and treatment response. 14/80 had infective aortitis: 11 had positive microbiology and 3 demonstrated empirical antibiotic response. Table 1 shows demographics. 12/80 had atheromatous disease. 18/80 were not further investigated due to comorbidity, lack of correlation with symptoms, or absence of follow-up. Initial imaging modalities with the highest yield were computed tomography (CTs) with aorta protocols and CTs of thorax/abdomen/pelvis. The most useful radiology report terms included: aortitis, periaortic inflammation, Takayasu’s, and hyperintense vessel uptake. Conclusion Our study is the first retrospective case analysis, to our knowledge, of more than 15,000 radiology reports used as a starting point to evaluate for aortitis, identifying a large dataset with a broad case-mix. It contrasts with current literature identifying cases histologically and post-operatively. We found that radiology can be a useful early alert for possible diagnoses which require further assessment. Consequently, we developed an alerting system within our radiology department based on the search terms and imaging modalities identified. This links to a multidisciplinary meeting including vascular and rheumatology, so highlighted cases are discussed early. Disclosures K. Townsend: None. G. Cattini: None. K. Moss: None. K. Stenson: None. P. Holt: None. R. Morgan: None. N. Sofat: None.

IDIOPATHIC AORTITIS: AN ATYPICAL CASE ISOLATED TO THE CORONARY SINUS LEADING TO OBSTRUCTIVE CORONARY ARTERY DISEASE

Aortitis encompasses a wide range of etiologies involving inflammation of the aorta. While most forms of aortitis can be linked to a specific cause, patients with idiopathic aortitis (IDA), are asymptomatic and usually diagnosed after surgical removal. 29 year old female with past medical history

287 Aortitis and Aortic Valvulitis from Autoimmune Non-Necrotising Vasculitis

A 45-year-old female presented with Acute pulmonary oedema associated with Severe Aortic regurgitation (AR) . Past medical history included long term immunosuppression with Hydroxychloroquine and Prednisolone for the management of Discoid Lupus with no previous systemic involvement. Trans-oesophageal Echocardiogram demonstrated thickened, fibrotic, tented aortic valve leaflets without central coaptation causing severe AR . There were no lesions typical of Libman-Sacks endocarditis and no appreciable masses or vegetations. Computed tomography coronary angiogram showed thickening around the aortic root with stenotic lesions in the coronary arteries and a zero-calcium score. Coronary angiogram confirmed these stenotic lesions. Fluorodeoxyglucose - Positron emission tomography showed increased uptake within the aortic root, ascending aorta, and proximal head and neck vessels .The findings were suggestive of vasculitis, however, a complete vasculitis screen was non-contributory. Mechanical Aortic Valve Replacement and Coronary Artery Bypass Grafting to the Right Coronary Artery and Left Anterior Descending Coronary Artery was performed. Histopathology of the aortic root/valve revealed non-necrotising aortitis (NNA), most likely representative of autoimmune associated aortitis. NNA is associated with autoimmune disease in approximately 50% of the cases with a slight female predominance and a typical age of presentation between 60-90 years. Differentials include Takayasu Aortitis, IgG4-related Aortitis, Syphilitic Aortitis, Systemic Lupus Erythematosus, and Isolated Idiopathic Aortitis. In this case, biopsy showed non-specific chronic aortitis with negative spirochete immunohistochemistry, scant IgG4 plasma cells, and no giant cells, with considerable overlap between various aetiologies. The history of immunosuppression treatment may have contributed to the modified histological appearance of aortitis.

EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice summary.

Large vessel vasculitis (LVV) is the most common form of primary vasculitis comprising of giant cell arteritis (GCA), Takayasu's arteritis (TAK) and idiopathic aortitis. Early diagnosis and treatment of LVV are paramount to reduce the risk of ischemic complications such as visual loss and strokes, vascular stenosis and occlusion, and aortic aneurysm formation. Use of imaging modalities [ultrasound (US), magnetic resonance imaging (MRI), computed tomography (CT) and [18F]-fluorodeoxyglucose positron emission tomography (PET)] has steadily increased to enable assessment of cranial and extracranial arteries, as well as the aorta. These imaging modalities are less invasive, more sensitive and readily available compared to temporal artery biopsy (TAB). Modern imaging methods have changed the role of TAB in diagnosing GCA and have replaced diagnostic angiography. Over the last two decades, several studies have evaluated the use of US, MRI, CT and PET in LVV. However, these various imaging tools are not yet uniformly used in routine clinical practice and controversy exists as to which imaging modality best provides meaningful assessments of disease activity and damage in LVV. In January 2018, evidence-based recommendations for the use of imaging modalities in LVV were published. The aim of this review is to summarize the current evidence of imaging in patients with or suspected of having LVV, and to highlight the clinical implications of the EULAR recommendations.

Nonatherosclerotic Abdominal Vasculopathies

Nonatherosclerotic abdominal arterial vasculopathies (NAVs), including mesenteric or renal artery dissection, aneurysm, stenosis, and vasculitis, are rare but have great clinical significance. Patients may present emergently with life-threatening complications such as arterial rupture and hemorrhagic shock. Herein, we present our center's experience with NAVs and provide extensive literature review to close the gap in the scarce, related literature. From a single-center retrospective data analysis, we identified and characterized subjects (aged 18-60 years) who presented with NAV between January 2000 and December 2015. Of the 1416 charts reviewed, 118 met inclusion criteria. The average age of patients with NAV was 47.0±9.9years, mostly affecting women (64%). Primary diagnoses included fibromuscular dysplasia (FMD) (25.4%), isolated aneurysms (24.6%), and median arcuate ligament syndrome (MALS) (15.3%). Less common diagnoses were localized vasculitis of the gastrointestinal tract (LVGT) (7.6%), isolated dissection (5.1%), microscopic polyangiitis and granulomatosis with polyangiitis (5.1%), trauma (4.2%), segmental arterial mediolysis (4.2%), Ehlers-Danlos syndrome (2.5%), Takayasu's arteritis (2.5%), polyarteritis nodosa (1.7%), idiopathic abdominal aortitis (0.8%), and Loeys-Dietz syndrome (0.8%). Females constituted 90% of patients with FMD, 77.8% with MALS, 77.8% with isolated aneurysms, 66.7% with Takayasu arteritis, and 55.6% with LVGT. Prevalent comorbidities included tobacco use (43.6%) and hypertension (52.1%). Coil embolization was used in 14.4%, anticoagulation in 11.9%, angioplasty/stenting in 11.9%, open resection/surgical revascularization in 10.2%, and prednisone in 10.2% of the cases. Conservative management was pursued in 33.1% of the patients. A high degree of symptom relief was shown in 91.7%. NAV are rare and can be caused by different etiologies that primarily affect females. Hypertension and tobacco use were prevalent. Various imaging strategies revealed aneurysms, stenosis, dissection, and/or thrombosis affecting renal and celiac arteries. Most patients improved with conservative, medical, endovascular, or surgical approach. More research is needed to standardize management approach to patients with NAV.

Idiopathic Inflammatory Ascending Aortitis Treated Successfully with Steroids

ABSTRACT Patients with aortitis, which can be infectious or inflammatory, may have findings that mimic the acute aortic pathology from an aortic dissection, intramural hematoma, or a penetrating aortic ulcer. A prompt determination of the etiology of aortic pathology via multimodal imaging and clinical assessment is critical to guiding care because treatment for each condition varies widely.

Diagnosis, Management, and Outcome of Aortitis at a Single Center.

Aortitis is a rare condition with inflammatory or infectious etiology that can be difficult to diagnose due to the highly variable clinical presentation and nonspecific symptoms. However, current literature on the diagnosis, management, and prognosis of aortitis is extremely scarce. We retrospectively reviewed all patients' charts who were diagnosed with giant cell arteritis, Takayasu arteritis, or noninfectious aortitis presenting at a single center between January 1, 2009, and April 17, 2015. Data collected included demographics, medical history, comorbidities, laboratory and imaging data, management, and outcome. Among the included 15 patients presenting with aortitis at our center, 53% were diagnosed with Takayasu arteritis, 33% with idiopathic inflammatory aortitis, and 13% with giant cell arteritis. All patients received steroid treatment, 67% received adjunctive immunosuppressants or immunomodulators, and 33% underwent interventional procedures. Based on clinical presentation and laboratory and imaging findings at the last follow-up visit for each patient, 67% showed improvement, 27% had no change in disease activity, and 7% had a progression of the disease. Takayasu arteritis was found to be more common than idiopathic inflammatory aortitis and giant cell arteritis among our 15 cases diagnosed with aortitis. All patients received medical therapy and 33% received interventional procedures, leading to 67% improvement of disease activity or related complications. This article also offers a comprehensive review of the diagnosis, management, and outcome of aortitis, supplementing the very limited literature on this disease.

Varicella Zoster Virus and Large Vessel Vasculitis, the Absence of an Association.

Objective:It is controversial whether microorganisms play a role in the pathogenesis of large and medium vessel vasculitides (eg, giant cell arteritis [GCA], Takayasu arteritis [TAK] and focal idiopathic aortitis [FIA]). Recent studies have reported the presence of Varicella Zoster Virus (VZV) within formalin-fixed, paraffin-embedded temporal arteries and aortas of about three-quarters or more of patients with these conditions, and in a minority of controls. In a prospective study, we sought to confirm these findings using DNA extracted from vessels that were harvested under surgically aseptic conditions and snap frozen.Methods and Results:DNA samples extracted from 11 surgically sterile temporal arteries and 31 surgically sterile thoracic aortas were used in an attempt to identify the vessel-associated VZV genome. Two different validated PCR methods were used. Thirty-one thoracic aorta aneurysm specimens included biopsies from 8 patients with GCA, 2 from patients with TAK, 6 from patients with FIA, and 15 from patients without vasculitis, who had non-inflammatory aneurysms. Eleven temporal artery biopsies were collected from 5 patients with GCA and 6 controls. The presence of VZV was not identified in either the specimens from patients with large vessel vasculitis or from the controls.Conclusions:Using surgically sterile snap-frozen specimens, we were unable to confirm recent reports of the presence of VZV in either aortas or temporal arteries from patients with large vessel vasculitis or controls.

Épidémiologie et histoire naturelle de l’artérite à cellules géantes (Horton)

Knowledge of the natural history and epidemiology of giant cell arteritis (GCA) is growing. With the recent conceptual change, GCA is no longer considered a disease with mandatory cranial symptoms but, rather, a larger disease spectrum also including idiopathic aortitis in people older than 50 and polymyalgia rheumatica with large-vessel involvement. The incidence peak between age 70 and 80 years, greater frequency in females and greater occurrence in Nordic countries are well-established epidemiological characteristics. Conversely, the notion that the incidence of GCA is increasing is challenged by several recent population-based studies suggesting a trend reversal for about 15 to 20 years. The known link with the allele HLA-DRB1*04 was confirmed by a genome-wide association study that also found associations with two other genetic polymorphisms. Recent studies indicating a link with varicella zoster virus infection have invigorated the hypothesis of an infectious cause for GCA. Smoking is the most solidly recognized environmental risk factor, but other traditional cardiovascular risk factors do not seem to predispose to GCA. Evidence is mounting that overall mortality in GCA patients is at best slightly higher than expected in relation to general population mortality data, but GCA is associated with an increase in morbidity and mortality specifically related to aortic aneurysm or other cardiovascular causes. Further studies are needed to integrate the current knowledge into a single etiological model.

A case series of surgically diagnosed idiopathic aortitis in a Canadian centre: a retrospective study.

Idiopathic aortitis became recognized relatively recently, and the body of knowledge concerning this condition is scarce. We aimed to determine the frequency of idiopathic aortitis in aortic specimens, the clinical, laboratory and radiologic characteristics at diagnosis and during follow-up, and the approach to investigation, treatment and monitoring taken by the treating physicians. We identified cases of aortitis diagnosed on pathological specimens of the aorta between Jan. 1, 2003, and July 31, 2013, at The Ottawa Hospital by reviewing the hospital's pathology database. Charts of identified patients were reviewed, and data on patient demographic characteristics, clinical features, laboratory and imaging tests, treatment and outcomes were analyzed. A total of 684 aortic specimens were analyzed during the study period; 47 cases of aortitis were identified, 32 of which were idiopathic. Twenty-one patients (66%) had complete imaging of branch vessels at baseline, 16 (76%) of whom had additional aortic or branch vessel lesions. Twelve patients (38%) received corticosteroids postoperatively. Over a mean follow-up period of 47.5 months, among the 12 patients (38%) who had complete imaging of branch vessels at least once, new aortic or branch lesions were diagnosed in 5 (42%); 3/32 patients (9%) required additional vascular surgery; and a new systemic condition was diagnosed in 2/32 (6%). Idiopathic aortitis is commonly discovered incidentally on examination of the pathological specimen following ascending aortic aneurysm repair. No guidelines exist for the investigation, treatment and follow-up of this condition, resulting in great variability of practice. Good-quality prospective studies are needed to address the many unanswered clinical questions regarding idiopathic aortitis and to allow formulation of more definitive recommendations.

Comparison of idiopathic (isolated) aortitis and giant cell arteritis-related aortitis. A French retrospective multicenter study of 117 patients.

The aim of the study was to compare clinical/imaging findings and outcome in patients with idiopathic (isolated aortitis, IA) and with giant cell arteritis (GCA)-related aortitis. Patients from 11 French internal medicine departments were retrospectively included. Aortitis was defined by aortic wall thickening >2mm and/or an aortic aneurysm on CT-scan, associated to inflammatory syndrome. Patients with GCA had at least 3 ACR criteria. Aortic events (aneurysm, dissection, aortic surgeries) were reported, and free of aortic events-survival were compared. Among 191 patients with non-infectious aortitis, 73 with GCA and 44 with IA were included. Patients with IA were younger (65 vs 70 years, p=0.003) and comprised more past/current smokers (43 vs 15%, p=0.0007). Aortic aneurisms were more frequent (38% vs 20%, p=0.03), and aortic wall thickening was more pronounced in IA. During follow-up (median=34 months), subsequent development of aortic aneurysm was significantly lower in GCA when compared to IA (p=0.009). GCA patients required significantly less aortic surgery during follow-up than IA patients (p=0.02). Mean age, sex ratio, inflammatory parameters, and free of aortic aneurism survival were equivalent in patients with IA ≥ 60 years when compared to patients with GCA-related aortitis. IA is more severe than aortitis related to GCA, with higher proportions of aortic aneurism at diagnosis and during follow-up. IA is a heterogeneous disease and its prognosis is worse in younger patients <60 years. Most patients with IA ≥ 60 years share many features with GCA-related aortitis.

Isolated Aortitis Presenting with an Annoying Persistent Cough: A Case Report.

ObjectivesTo report a case of idiopathic aortitis, presenting with chronic cough.Materials and MethodsThe Authors describe the case of a 72-year-old man with dry cough, worsening fatigue, weight loss and elevated systemic inflammatory markers.ResultsA PET-CT scan showed diffuse thickening of the thoracic aorta and confirmed the diagnosis of aortitis. Systemic corticosteroid therapy was initiated and complete remission was achieved in six months.ConclusionPersistent dry cough of unknown origin, especially when associated with systemic inflammation, demands a thorough differential diagnosis and should not be underrated.LEARNING POINTSIsolated aortitis may produce unrevealing symptoms, which are often dismissed.Chronic unproductive cough should raise a suspicion of isolated aortitis.The critical diagnostic exam for aortitis is a PET-CT scan.High-dose corticosteroids with gradual tapering, lead the disease remission.

A Case of Idiopathic Aortitis with Left Renal Vein Thrombosis

A 38-year-old man was admitted to the hospital because of abrupt left flank pain. He had no fever and physical examination revealed tenderness of the left costovertebral angle. Laboratory data revealed white blood cell 16,060/µL, C-reactive protein 0.93 mg/ dL. Urinalysis showed more than 1/2 red cells per high-power field with severe proteinuria (4+). Enhanced computed tomography (CT) showed the thickened abdominal aorta wall with partial thrombus. The thickened aorta wall compressed the left renal vein and it caused left renal vein thrombosis. Abdominal CT findings suggested aortitis of the abdominal aorta with complication of left renal vein. We could exclude other types of aortitis including autoimmune aortitis, Takayasu’s arteritis, giant cell arteritis, and infectious causes based on a serologic test and the history of the patient. Therefore, the patient was diagnosed with idiopathic aortitis and treated with glucocorticoid. After treatment, his symptoms disappeared and a follow-up CT showed decreased mural thickening of the abdominal aorta. Isolated idiopathic aortitis presented with renal vein thrombosis is extremely rare and has not been reported in Korea yet. We present a rare case report on idiopathic aortitis of the abdominal aorta with complication of left renal vein thrombosis.

Clinical Diagnosis and Management of Large Vessel Vasculitis: Giant Cell Arteritis

Large vessel vasculitis (LVV) covers a spectrum of primary vasculitides predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA) and Takayasu arteritis (TA). Less commonly LVV occurs in various other diseases. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Occasionally LVV is discovered unexpectedly on pathological examination of a resected aortic aneurysm. Clinical evaluation is often unreliable in determining disease activity. Moreover, the diagnostic tools are imperfect. Acute phase reactants can be normal at presentation and available imaging modalities are more reliable in delineating vascular anatomy than in providing reliable information on degree of vascular inflammation. Glucocorticoids are the mainstay of therapy of LVV. Patients may develop predictable adverse effects from long-term glucocorticoid use. Several steroid-sparing agents have also shown some promise and are currently in use. Endovascular revascularization procedures and open surgical treatment for aneurysms and dissections are sometimes necessary, but results are not always favorable and relapses are common. This article, the first in a series of two, will be devoted to GCA and isolated (idiopathic) aortitis, while TA will be covered in detail in the next article.

AB0589 Non-Infectious Aortitis: Still an Underdiagnosed Entity. Report of 32 Cases from A Single Center

BackgroundNon infectious aortitis may present as an idiopathic isolated condition or associated with a wide spectrum of diseases. Aortitis often presents with nonspecific symptoms leading in many cases to an...

Frequency of immunoglobulin G4‐related aortitis in cases with aortic resection and their clinical characteristics compared to other aortitises

To identify the frequency of immunoglobulin G4 (IgG4)-related aortitis in patients who undergo aorta surgery and are diagnosed by pathology as having chronic aortic inflammation and to compare IgG4-related aortitis with other non-infectious aortitises in terms of clinical characteristics. The aorta specimen pathological reports of 1418 patients who underwent aortic aneurysm or dissection surgery were reviewed. In total, 41 had chronic aortic inflammation without atherosclerosis, cancer or infection. Their aorta biopsy specimens were subjected to IgG4 immunostaining. IgG4-related aortitis was diagnosed if the IgG4-positive plasma cell count exceeded 50 per high power field (HPF), the ratio of IgG4-positive to IgG-positive plasma cells exceeded 50% and dense lymphoplasmacytic infiltration, fibrosis and/or obliterative phlebitis were observed. Of the 41 non-infectious aortitis cases, 29, six and six had idiopathic aortitis, Takayasu's arteritis and Behcet's aortitis, respectively. Of the 29 idiopathic aortitis cases, three had IgG4-related aortitis. All were male and > 65 years of age. Two had thoracic aortic aneurysms and one had an abdominal aortic aneurysm. Their IgG4-positive plasma cell counts were 60/HPF or higher; lymphoplasmacytic infiltration and/or fibrosis, but not obliterative phlebitis, were observed. The IgG4-related aortitis cases were older (67 [range, 65-69] years) than the Takayasu's arteritis (47.5 [38-58] years) or Behcet's aortitis (47 [31-56] years) cases and more likely to be male than the Takayasu's arteritis cases (100% vs. 17%). In patients with chronic aortic inflammation, 7% had IgG4-related aortitis. This disease may be more common in older male patients than in other demographic groups.

Role of ultrasound in the understanding and management of vasculitis

Vasculitis is characterized by a circumferential vessel-wall thickening ('halo'), which can be visualized by modern imaging techniques. In particular, the resolution of ultrasound has increased to 0.1 mm. Ultrasound detects abnormalities that are pathognomonic even in arteries with a diameter below 1 mm. It is particularly helpful in the diagnosis of large-vessel vasculitides, such as classic temporal arteritis, large-vessel giant-cell arteritis (GCA), Takayasu arteritis and idiopathic aortitis. Echocardiography is important for determining cardiac involvement in Takayasu arteritis and also for examining the coronary arteries of children with suspected Kawasaki disease, which is a medium-vessel vasculitis. In small vessel vasculitides ultrasound has only a role for determining the distribution or organ involvement. Fast-track clinics for the diagnosis of GCA help to initiate treatment before complications such as blindness occur; patients receive appointments within 24 h in these clinics. Clinical examination and ultrasound of temporal and axillary arteries are performed by an experienced rheumatologist. In most cases this is able to determine if GCA is present. Temporal artery biopsy can be still carried out in ambivalent cases. The wall swelling of temporal arteries disappears after 2-3 weeks of glucocorticoid treatment. After 3 days of treatment, diagnosis becomes more difficult with ultrasound in some cases. In larger arteries, such as the axillary arteries, wall thickening disappears within months. It tends to be darker (more hypoechoic) in acute disease because of oedema.

CT angiographic imaging characteristics of thoracic idiopathic aortitis

BackgroundIdiopathic aortitis (IA) is characterized by giant cell or lymphoplasmacytic inflammation of aorta without a secondary cause. ObjectiveWe undertook a retrospective case–control study to identify characteristic CT angiographic findings in these patients and to correlate them with known atherosclerotic risk factors. MethodsIA cases and controls with noninflammatory aneurysm (control group I) and patients with secondary aortitis (control group II) were identified with a pathology database. Preoperative CT angiographic images of thoracic aorta were reviewed. Diameter of thoracic aorta, wall thickness, and calcification were measured at various sites. Traditional atherosclerotic risk factors were identified from case records and included hypertension, hyperlipidemia, diabetes mellitus, and smoking. ResultsTwenty-two idiopathic aortitis cases were compared with 18 patients in control group I and 16 patients in control group II. No differences were found in prevalence of hypertension and diabetes, but hyperlipidemia was more prevalent in the control group I than in cases (72% vs 36%; P = .03). Current smoking was more prevalent in cases (24%) than for patients in control group I (6%) and group II (19%) but not statistically significant (P = .18 and .69, respectively). Thoracic aortic diameters at various points were significantly larger in cases than for patients in control group I. Calcification was more frequent in cases than for patients in control group II. No differences in wall thickness were found. No meaningful correlation was observed between atherosclerotic risk factors and aortic diameter and calcification scores. ConclusionsPatients with IA have significantly larger and more diffuse dilatation of the thoracic aorta than patients with noninflammatory aneurysms.

AB0464 Biologic therapy in aortitis: a multicenter study of 30 patients

BackgroundAortitis is the inflammation of the aortic wall, regardless of the underlying condition. It can occur alone or associated with other causes. Aortitis is often refractory to standard immunosuppressive therapy.ObjectivesTo...

Imaging in vasculitis

Vasculitides may involve small, medium-sized or large arteries. In small-vessel vasculitides imaging studies of lungs, nasal sinuses, cerebrum, heart and other organs are important for determining disease extension and disease activity. Aneurysms are a hallmark of medium-sized artery vasculitides. In large-vessel vasculitis, imaging studies depict homogeneous, circumferential wall swelling and smoothly tapered luminal narrowing. Ultrasound and high-resolution magnetic resonance imaging (MRI) show characteristic wall abnormalities in temporal and occipital arteries whereas ultrasound, MRI, MR angiography, computed tomography (CT), CT angiography and positron emission tomography delineate characteristic features in extra-cranial arteries that are affected in large-vessel giant cell arteritis, Takayasu arteritis and idiopathic aortitis. Conventional angiography has still its place for therapeutic interventions. Imaging has led to a better understanding of the nature and distribution of vasculitides. It significantly facilitates diagnosis of patients with suspected vasculitis.