Extracranial Giant Cell Arteritis Research Articles

49. Giant cell arteritis and Takayasu arteritis: two sides of the same coin in an elderly female?

49. Giant cell arteritis and Takayasu arteritis: two sides of the same coin in an elderly female?

Giant-cell Arteritis of the Ovarian Arteries: A Rare Manifestation of a Common Disease.

We describe a 58-year-old woman presenting with headache and an elevated erythrocyte sedimentation rate (ESR), who was diagnosed with and successfully treated for giant-cell arteritis (GCA). Seven months after the end of treatment, ovarian GCA was incidentally found after ovariectomy for a simple cyst. GCA of extracranial vessels like the ovarian arteries is rare. Nevertheless, we stress that extracranial GCA should be considered in patients older than 50 years with an elevated ESR, even if a temporal artery biopsy is negative or specific symptoms are absent. Moreover, we discuss the importance of imaging techniques when GCA of the extracranial large vessels is suspected.LEARNING POINTSAlthough rare, ovarian arteries can be involved in giant-cell arteritis (GCA).Extracranial GCA should be considered in the differential diagnosis of patients aged 50 years or older with an elevated ESR, even if temporal artery biopsy is negative or specific symptomatology of GCA is absent.If GCA is suspected but the origin is unclear, an MRA or PET-CT scan should be performed to screen for GCA of extracranial arteries.

Clinically isolated aortitis: pitfalls, progress, and possibilities.

Non-infectious aortitis may be caused by several distinct systemic rheumatologic diseases. In some patients, aortitis is identified either pathologically or radiologically in the absence of clinical evidence of a systemic vasculitis. By consensus nomenclature, such cases are referred to as clinically isolated aortitis (CIA). Some systemic disorders may initially present as CIA including giant cell arteritis (GCA), IgG4-related disease, infectious aortitis, and granulomatosis with polyangiitis. CIA most commonly occurs in women of European descent over the age of 50 and, thus, mirrors the gender, age, and geographic distribution of GCA. CIA most often demonstrates a granulomatous/giant cell pattern of inflammation (GPI), and CIA-GPI is pathologically indistinguishable from aortitis due to GCA. In many cases, CIA may be a manifestation of extracranial GCA. CIA is being identified both pathologically in resected aortic tissue and radiologically by computed tomography scanning, magnetic resonance imaging, and fluorodeoxyglucose positron emission tomography. However, there appears to be significant differences between pathologically defined CIA and radiologically defined CIA. Multiple studies have shown that patients with CIA are at increased risk for subsequent aortic events (new aneurysms or dissections) and thus it is recommended to monitor these patients with periodic aortic imaging. While the data is currently limited, there is increasing evidence that at least some patients with CIA may benefit from immunosuppressive therapy.

020 A Case of Extensive Extracranial Giant Cell Arteritis Presenting with a Stroke

020 A Case of Extensive Extracranial Giant Cell Arteritis Presenting with a Stroke

Broadening the differential diagnosis in extracranial giant cell arteritis

Infective endocarditis was an important differential diagnosis in the reported case where clinical stigmata of extracranial giant cell arteritis (GCA) included the association of a systolic murmur and splinter haemorrhages.1 Infective endocarditis was even more strikingly simulated by GCA in a 64-year-old woman who presented with pyrexia, headache, neck ache and a systolic murmur, and …

Impact of cranial and axillary/subclavian artery involvement by color duplex sonography on response to treatment in giant cell arteritis

Color duplex sonography (CDS) today is broadly used in the diagnostic workup of patients with suspected cranial or extracranial giant cell arteritis (GCA). This study aimed to determine the prognostic impact of the disease pattern assessed by CDS on the treatment response in GCA. This was a retrospective, longitudinal follow-up study of 43 patients who were diagnosed with GCA at our institution between 2002 and 2010. All patients underwent CDS of the temporal and subclavian/axillary arteries at baseline and were observed for at least 6 months. Vasculitis was sonographically characterized by a circumferential, hypoechogenic wall thickening. According to the CDS findings, patients were categorized into patients with involvement of the subclavian/axillary arteries only (group A1, n = 17), patients with involvement of both the subclavian/axillary arteries and the temporal arteries (group A2, n = 9), and patients with isolated cranial GCA (group B, n = 17). Data on recurrences, corticosteroid doses, and steroid-sparing agents were extracted from the medical records. Treatment response over time was analyzed by Kaplan-Meier curves with log-rank testing. The mean follow-up time was 25.4 months and did not differ between groups (P = .4). Patients in group A1 were significantly younger than patients in groups A2 and B (P < .01). The interval between symptom onset and diagnosis was significantly longer in groups A1 and A2 compared with group B (P < .01). The number of recurrences per month was significantly higher in group A2 compared with group A1 and group B (A1, 0.07; A2, 0.13; B, 0.03; P < .01). Whereas there were no significant differences in the mean time until a daily prednisolone dose <10 mg was reached, patients in group A2 more frequently required steroid-sparing agents (A1, 24%; A2, 56%; B, 24%; P = .04). Extensive vascular involvement of both the temporal and subclavian/axillary arteries, as depicted by CDS, may be associated with a poor treatment response in GCA.

Extra-cranial giant cell arteritis and Takayasu arteritis: How similar are they?

ObjectiveTo compare clinical and imaging characteristics of patients with giant cell arteritis (GCA) and upper extremity (UE) arterial involvement to patients with Takayasu arteritis (TAK). MethodsA cohort of patients seen at the Mayo Clinic with TAK diagnosed between 1984 and 2009 and a cohort of patients with GCA and UE arterial involvement diagnosed between 1999 and 2008 were studied. ResultsThe TAK cohort consisted of 125 patients (91% female); the mean age (±SD) at diagnosis was 30.9 (±10) years. The cohort of patients with GCA and UE involvement comprised of 120 patients (80% female); the mean age (±SD) at diagnosis was 67.8 (±7.5) years. The mean time from onset of symptoms to diagnosis was significantly longer in TAK (3.2 years) than GCA (0.5 years), p < 0.001. UE claudication was reported in 40% with TAK and 53% with GCA, p = 0.04. UE blood pressure discrepancy was present in 65% with TAK versus 28% with GCA, p < 0.001. Involvement of the thoracic aorta, abdominal aorta, carotid arteries, innominate artery, mesenteric artery, and left renal artery was more frequently observed in TAK (p < 0.05). Among patients with luminal changes of the thoracic aorta, stenotic/occlusive lesions were predominant in TAK (81% compared to 0% in GCA), whereas aneurysmal disease was more common in GCA (100% compared with 19% in TAK, p < 0.001). ConclusionPatients with GCA and UE involvement differ from patients with TAK in clinical and imaging characteristics. Aortic aneurysms were more common in GCA, while stenotic changes of the aorta were more common in TAK, suggesting different pathophysiologic mechanisms or vascular response to injury.

Towards an optimal semiquantitative approach in giant cell arteritis: an 18F-FDG PET/CT case-control study

Giant cell arteritis (GCA) is the most common form of vasculitis in western countries. (18)F-FDG PET has been shown to be a valuable tool for the diagnosis of extracranial GCA, but results of studies are inconsistent due to a lack of standardized (18)F-FDG PET criteria. In this study, we compared different semiquantitative approaches using a controlled design to define the most efficient method. All patients with biopsy-proven GCA who had undergone an (18)F-FDG PET/CT scan in our PET unit were reviewed and matched with a control group based on age and sex. Different semiquantitative arterial (ascending and descending thoracic aorta and aortic arch) to background (liver, lung and venous blood pool) SUV ratios were blindly compared between GCA patients and matched controls. We included 11 patients with biopsy-proven GCA cases and 11 matched controls. There were no differences between the groups with regard to body weight, injected radioactivity, blood glucose level or CRP. The arterial to venous blood pool ratios discriminated the two groups better than other methods when applied to the aortic arch and the descending thoracic aorta (p<0.015). In particular, the highest aortic to highest blood pool SUVmax ratio, when applied to the aortic arch, provided optimal diagnostic performance (sensitivity 81.8 %, specificity 91 %, AUC 0.87; p<0.0001) using a cut-off value of 1.53. Among all tested (18)F-FDG PET/CT methods, the aortic to blood pool SUVmax ratio outperformed the liver and lung ratios. We suggest the use of this ratio for the assessment of aortic inflammation in GCA patients.

Extra-cranial giant cell arteritis: early and late consequences

Extra-cranial giant cell arteritis: early and late consequences

Dr. Czihal replies: Figure 1.

To the Editor: We appreciate the comments from Milchert, et al 1 on our study evaluating the involvement of femoropopliteal arteries in giant cell arteritis (GCA)2. In their letter, they describe 2 cases with different clinical manifestations of extracranial GCA, including 1 patient with suspected vasculitis of the lower extremities. They discuss the difficulties in discriminating between vasculitis and arteriosclerosis in clinical practice. Detection of a homogenous, hypoechogenic, circumferential vessel wall thickening by color duplex sonography (CDS) has been shown to be specific for …

Sonographic and clinical pattern of extracranial and cranial giant cell arteritis

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.

Magnetic Resonance Angiography in Extracranial Giant Cell Arteritis

Noninvasive diagnosis of giant cell arteritis (GCA) remains challenging, particularly with regard to evaluation of extracranial arterial disease. The objective of the study was to retrospectively review extracranial involvement in patients with GCA and/or polymyalgia rheumatica (PMR), evaluated with magnetic resonance imaging (MRI), especially 3-dimensional contrast-enhanced magnetic resonance angiography images of the aortic arch and its branches. Clinical information, biopsy status, and MRI examinations of 28 patients with GCA/PMR were reviewed. Patient images were mixed randomly with 20 normal control images and were independently reviewed by 2 radiologists. Interobserver agreement for detection of arterial stenosis was determined by the k coefficient. Both readers described vascular alterations in keeping with extracranial GCA in 19 of 28 patients (67%) with good interobserver agreement (k = 0.73) and with even higher agreement on diagnosing nonocclusive versus occlusive disease (k = 1.00). The most common lesions were bilateral axillary stenosis or obstructions, observed by both readers in 8 patients (28%). Among the 19 patients with magnetic resonance angiography lesions in the subclavian/axillary arteries, 12 (75%) had biopsy-proven GCA, but only 5 (41%) of these patients had clinical features of large artery disease. In our series review, MRI could provide accurate information on involvement of the aortic arch and its branches in extracranial GCA, depicting different degrees of stenosis. Our analysis also illustrates that occult large artery vasculitis should be considered in patients without biopsy-proven GCA, patients with classic GCA but without clinical signs of large artery disease, and in patients initially diagnosed as having PMR.

Balloon angioplasty of arteries of the upper extremities in patients with extracranial giant-cell arteritis

Balloon angioplasty of arteries of the upper extremities in patients with extracranial giant-cell arteritis

Clinical presentation and vascular imaging in giant cell arteritis of the femoropopliteal and tibioperoneal arteries. Analysis of four cases

We present four patients with rapidly progressive claudication of the lower limbs due to extracranial giant cell arteritis. Additional findings suggestive of giant cell arteritis were involvement of the axillary or brachial arteries in two patients, symptoms of polymyalgia rheumatica or temporal arteritis in three, and all patients had severely elevated erythrocyte sedimentation rate and C-reactive protein level. Lower limb involvement affected preferentially the femoropopliteal, deep femoral, and tibioperoneal arteries. Hypoechogenic, concentric mural thickening suggestive of vasculitis was readily visible in all involved arterial segments by duplex ultrasound imaging, whereas angiography was rather unspecific. Typical changes for large-vessel vasculitis were also detectable by magnetic resonance imaging and fluorine-18-desoxyglucose positron emission tomography. More widespread use of these vascular imaging techniques may show that giant cell arteritis of the lower limbs is more frequent than previously assumed.

Uncommon arteriopathies: what the vascular surgeon needs to know

Uncommon arteriopathies encompass a diverse range of diseases, including inherited collagen vascular disorders such as Marfan syndrome, Ehlers-Danlos Type IV, and pseudoxanthoma elasticum; vasculitides, including Takayasu’s arteritis, extracranial giant cell arteritis, and Behçet’s disease; neurofibromatosis type 1; intimal fibromuscular dysplasia; unusual bacterial and viral infections; and drug-induced arteriopathies. Patients with uncommon arterial disorders may present to the vascular surgeon with common surgical problems, including intermittent claudication, renovascular hypertension, Raynaud’s phenomenon, and aneurysmal disease. However, the disease manifestations, expected course, and outcomes may be much different than more common arterial disorders and this can have important surgical implications. This review centers around several interesting cases and the differential diagnoses that should be considered when encountering an unusual clinical presentation. Reference is made to the literature for diagnostic criteria, clinical pearls, and how to avoid pitfalls in the evaluation and management of patients with unusual arteriopathies.

Color duplex ultrasonography in large-vessel giant cell arteritis: CASE REPORT

In a patient with active extracranial giant cell arteritis, duplex ultrasonography demonstrated hypoechoic mural thickening of the brachial, axillary, subclavian, and carotid arteries with bilateral subtotal occlusions of the brachial and axillary arteries. The ultrasound image of the artery walls became midechoic within 8 weeks, and hyperechoic within one year after start of treatment with corticosteroids. A similar hypoechoic mural thickening of the temporal arteries has been recently described in active giant cell arteritis. The dark ultrasound image is due to an edema of the vessel wall in the acute stage. The brighter ultrasound image might be due to fibrosis in the chronic stage of the disease.

Aortic and extracranial large vessel giant cell arteritis: A review of 72 cases with histopathologic documentation

Giant cell arteritis (GCA) is closely identified with the temporal arteritis-polymyalgiarheumatica syndrome of the elderly. It is also a systemic disease that can cripple and kill. Up to 15% of patients with temporal arteritis may have angiographic evidence of extracranial GCA, and aortic insufficiency, ruptured aortic aneurysm, aortic dissection, stroke, or myocardial infarction may be the initial manifestation of systemic GCA. A review of 72 cases of aortic and extracranial GCA, all with histopathologic verification of the disease, revealed that 25% of patients with aortic and extracranial large-vessel GCA had asymptomatic temporal arteritis; the ascending aorta and aortic arch were most frequently involved (39%), followed by the subclavian and axillary arteries (26%), and the femoropopliteal arteries (18%). Nine patients (12.5%) underwent an upper or lower limb amputation. Of the 18 patients whose death was directly attributable to extracranial GCA the causes were ruptured aortic aneurysm (6), aortic dissection (6), stroke (3), and myocardial infarction (3). The findings of these 72 cases caution against attributing all aortic and large-vessel arterial disease in the elderly to atherosclerosis and emphasize that timely surgical intervention may be necessary for life-saving and limb-salvage in patients with aortic and extracranial GCA.

Generalized Giant-Cell Arteritis With Coronary Artery Involvement and Myocardial Infarction

Giant-cell arteritis (GCA) is a disease that may affect more than 1% of the elderly white population. Extracranial GCA, usually involving the aorta and its major branches, has been reported to be clinically detectable in 10% to 15% of patients with GCA. 1 When large vessels are involved, GCA may be life threatening because of aortic dissection, ruptured aortic aneurysm, and, more rarely, myocardial infarction. Despite these complications, the survival rate is not usually considered to be reduced when compared with the general population of the same age. 2 Report of a Case. —An 82-year-old white woman was referred to the neuro-ophthalmology service complaining of visual loss in the left eye of 2 days' duration. She had not felt well for the previous several months, noting headaches, difficulty chewing food, and arthralgias. An examination revealed visual acuity of 20/20 OD and 20/25 OS, with superior altitudinal visual field loss and