Objectives: The aim of our study was to describe the sonographic pattern and clinical manifestations of extracranial (i.e. carotid and proximal arm arteries) and cranial arterial involvement in patients with giant cell arteritis (GCA). Methods: One hundred and ten consecutive patients with an established diagnosis of GCA between January 2002 and June 2010 were identified retrospectively from a database. All patients underwent colour duplex sonography (CDS) of the superficial temporal, carotid, and proximal arm arteries at the time of diagnosis. Circumferential, homogeneous, hypoechogenic wall thickening was regarded as a typical sign for GCA. Sonographic and clinical characteristics of patients with and without extracranial vessel involvement were compared. Results: Extracranial GCA was observed in 59 of 110 subjects (53.6%). The axillary artery (48.2%) was most frequently affected and bilateral vessel involvement was present in almost all patients (94.8%). Compared to patients with cranial GCA, patients with extracranial GCA were significantly younger, frequently did not meet the American College of Rheumatology (ACR) criteria for classification of cranial GCA, exhibited a lower rate of permanent visual impairment, and were diagnosed later after onset of clinical symptoms (all p < 0.01). With increasing age, a continuous shift from GCA with extracranial arterial involvement to cranial GCA was observed. Conclusion: Using CDS, extracranial GCA is a common finding, most frequently observed in the axillary arteries. The clinical pattern of GCA with extracranial arterial involvement differs from that of cranial GCA.

Objective.: To determine the extent and clinical significance of giant cell arteritis (GCA) of the femoropopliteal arteries.Methods.: This was a retrospective clinical color duplex sonography (CDS) study; 60 of 112 consecutive patients with the diagnosis of GCA underwent complete clinical examination of the lower extremities including the vasculature, systolic ankle pressure measurement, and CDS scans of the femoropopliteal arteries within 1 year after diagnosis of GCA. Circumferential, hypoechogenic, homogenous wall thickening was regarded as a hallmark of femoropopliteal GCA.Results.: GCA of femoropopliteal arteries was present in 32 (53.3%) of 60 patients. In general, femoropopliteal GCA developed bilaterally (100%) and 14 patients (23.3%) had significant lower extremity artery obstructions secondary to vasculitis, all leading to symptomatic lower extremity ischemia, with development of critical leg ischemia in 4 patients. Compared with subjects without lower extremity vasculitis, patients with femoropopliteal involvement had a significant time delay until diagnosis (mean 23.9 vs 11.1 weeks; p = 0.03) and a higher frequency of concomitant vasculitis of the arm arteries (74.2% vs 42.9%; p = 0.02).Conclusion.: Femoropopliteal artery involvement appears to be a clinically relevant manifestation of GCA, frequently leading to symptomatic lower extremity ischemia. CDS of the femoropopliteal arteries is a noninvasive diagnostic tool for detection of lower extremity vasculitis in GCA.

Nowadays noninvasive vascular imaging has an important role in the diagnostic work-up of the large vessel vasculitides (LVV), most importantly giant cell arteritis (GCA) and Takayasu arteritis. Among the imaging modalities available, ultrasound (US) has several important advantages, including low costs, rapid and repetitive availability without exposure to radiation, and high spatial resolution for assessment of large and medium-sized arteries. Therefore, US can be regarded the first line imaging method in suspected LVV. In patients with suspected GCA, US can replace temporal artery biopsy in certain clinical scenarios, and the application of US early in the diagnostic work-up of suspected GCA in specialized fast track clinics has been suggested to reduce the rate of visual ischaemic complications and associated costs. In other LVV such as Takayasu arteritis and chronic periaortitis, the diagnostic accuracy in comparison to other noninvasive imaging methods has not been formally tested but can be considered to be excellent. However, quality of US is highly dependent on the operator’s experience, and assessment of the thoracic aorta which is frequently involved in GCA and TA is limited. The role of US in the follow-up of LVV under treatment is unclear. In view of the promising data supporting its value on the one hand and several uncertainties and controversies on the other hand, the present review article provides a comprehensive overview on current evidence for the application of US in the diagnosis and follow up of LVV. Recent multicentre study results and emerging trends such as the application of compression sonography in the diagnosis of GCA and the use of contrast enhanced ultrasound in disease activity assessment in Takayasu arteritis are discussed.

Dealing with vascular compression syndromes is one of the most challenging tasks in Vascular Medicine practice. This heterogeneous group of disorders is characterised by external compression of primarily healthy arteries and/or veins as well as accompanying nerval structures, carrying the risk of subsequent structural vessel wall and nerve damage. Vascular compression syndromes may severely impair health-related quality of life in affected individuals who are typically young and otherwise healthy. The diagnostic approach has not been standardised for any of the vascular compression syndromes. Moreover, some degree of positional external compression of blood vessels such as the subclavian and popliteal vessels or the celiac trunk can be found in a significant proportion of healthy individuals. This implies important difficulties in differentiating physiological from pathological findings of clinical examination and diagnostic imaging with provocative manoeuvres. The level of evidence on which treatment decisions regarding surgical decompression with or without revascularisation can be relied on is generally poor, mostly coming from retrospective single centre studies. Proper patient selection is critical in order to avoid overtreatment in patients without a clear association between vascular compression and clinical symptoms. With a focus on the thoracic outlet-syndrome, the median arcuate ligament syndrome and the popliteal entrapment syndrome, the present article gives a selective literature review on compression syndromes from an interdisciplinary vascular point of view.

Purpose To assess the diagnostic value of intima media thickness measurements and contrast-enhanced ultrasound (CEUS) of the supraaortic arteries in the assessment of disease activity in Takayasu arteritis (TA). Materials and Methods Patients with TA and involvement of the carotid and/or subclavian/axillary arteries underwent CEUS imaging in addition to B-mode and color duplex ultrasound. The maximum IMT (mIMT) was measured and high-resolution CEUS of the most prominently thickened vessel segment was performed. Two blinded readers reviewed the CEUS movies, with semiquantitative assessment of microbubble enhancement of the arterial wall (grade 0: no or minimal; grade 1: moderate; grade 2: pronounced). Clinical symptoms, acute phase reactants, and established indices of clinical disease activity (NIH criteria, ITAS score) were recorded. Results 40 examinations in 17 patients were analyzed. According to clinical judgement, 27 and 13 cases were classified as clinically inactive and active, respectively. An mIMT-cutoff of > 2.7 mm identified active disease with a sensitivity and specificity of 69.2 % and 88.9 %, respectively (area under the curve 0.83). The interobserver agreement of CEUS analysis was substantial (Cohen’s kappa 0.76). By consensus reading, 17, 15, and 8 cases were classified as uptake grade 0, grade 1 and grade 2, respectively. Grade 0 uptake was exclusively present in 17 clinically inactive patients. Grade 1 uptake was seen in 10 patients with clinically inactive disease and 5 patients with clinically active disease. Grade 2 uptake was exclusively present in 8 patients with active disease. Conclusion Both sonographic IMT measurements and high-resolution CEUS are promising in the ad-hoc assessment of disease activity in patients with TA.