Complication Of Giant Cell Arteritis Research Articles

Tocilizumab use in giant cell arteritis: comparing Pharmaceutical Benefits Scheme eligible and ineligible cases in South Australia.

Tocilizumab (TCZ) is increasingly used as a steroid-sparing agent in giant cell arteritis (GCA), but there are strict Pharmaceutical Benefits Scheme (PBS) restrictions for its use in Australia. Patients who do not meet the PBS criteria can obtain TCZ through public hospital individual patient use (IPU) schemes which may not be universally accessible. We compared patients receiving IPU-approved TCZ with patients receiving PBS-subsidised TCZ and found IPU approvals were granted mainly for visual loss, a serious complication of GCA, in patients who otherwise failed to meet PBS criteria. Further studies demonstrating that TCZ is comparatively more effective than prednisolone monotherapy, as well as cost-effective, are needed to substantiate the rationale for expanding PBS approval criteria.

Combined Orbital and Cranial Vessel Wall Magnetic Resonance Imaging for the Assessment of Disease Activity in Giant Cell Arteritis.

Acute visual impairment is the most feared complication of giant cell arteritis (GCA) but is challenging to predict. Magnetic resonance imaging (MRI) evaluates orbital pathology not visualized by an ophthalmologic examination. This study combined orbital and cranial vessel wall MRI to assess both orbital and cranial disease activity in patients with GCA, including patients without visual symptoms. Patients with suspected active GCA who underwent orbital and cranial vessel wall MRI were included. In 14 patients, repeat imaging over 12 months assessed sensitivity to change. Clinical diagnosis of ocular or nonocular GCA was determined by a rheumatologist and/or ophthalmologist. A radiologist masked to clinical data scored MRI enhancement of structures. Sixty-four patients with suspected GCA were included: 25 (39%) received a clinical diagnosis of GCA, including 12 (19%) with ocular GCA. Orbital MRI enhancement was observed in 83% of patients with ocular GCA, 38% of patients with nonocular GCA, and 5% of patients with non-GCA. MRI had strong diagnostic performance for both any GCA and ocular GCA. Combining MRI with a funduscopic examination reached 100% sensitivity for ocular GCA. MRI enhancement significantly decreased after treatment (P < 0.01). In GCA, MRI is a sensitive tool that comprehensively evaluates multiple cranial structures, including the orbits, which are the most concerning site of pathology. Orbital enhancement in patients without visual symptoms suggests that MRI may detect at-risk subclinical ocular disease in GCA. MRI scores decreased following treatment, suggesting scores reflect inflammation. Future studies are needed to determine if MRI can identify patients at low risk for blindness who may receive less glucocorticoid therapy.

Challenges and perspectives for the treatment of giant cell arteritis

Early diagnosis and prompt initiation of treatment are crucial to avoid severe complications in giant cell arteritis (GCA). The European Alliance of Associations for Rheumatology (EULAR) recommendations for the use of imaging in large vessel vasculitis have helped better define the role of different techniques for diagnosing and monitoring the disease. Regarding the treatment, corticosteroids remain the standard, and tocilizumab is the preferred corticosteroid-sparing treatment. New corticosteroid-sparing treatments and "ultra-light" corticosteroid usage regimens are also under study and could represent valid therapeutic alternatives in the future.

Giant cell arteritis associated with scalp, tongue or lip necrosis: A French multicenter case control study

BackgroundScalp, tongue and/or lip necrosis are rare complications of GCA. ObjectivesTo describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis. MethodsA retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period. Logistic regression analyses were conducted to identify baseline characteristics associated with scalp, tongue and/or lip necrosis. ResultsCompared to controls, patients with scalp, tongue and/or lip necrosis showed significantly more cranial manifestations (headache, p=0.045; scalp tenderness, p=0.006; jaw claudication, p=0.02). No differences were observed between both groups regarding the occurrence of visual symptoms or large vessel involvement. At diagnosis, GCA patients with necrosis more likely received IV methylprednisolone infusions and higher doses of oral prednisone. There were no differences regarding vascular complications during follow up. Compared to controls, survival was decreased in GCA patients with necrosis (p=0.003). In a multivariable logistic regression model, scalp tenderness [odds ratio (OR) 4.81(95 % CI: 1.57, 14.79), p = 0.006] and cognitive disorder [OR 6.42 (95 % CI: 1.01, 40.60), p=0.048] were identified as factors associated to scalp, tongue, and/or lip necrosis. ConclusionOur results suggest that scalp, tongue, and/or lip necrosis is associated to higher mortality in GCA patients. Scalp tenderness and cognitive disorder were significant factors associated to this very rare complication of GCA.

Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy?

Stroke is a rare and severe complication of giant cell arteritis (GCA). Although early diagnosis and treatment initiation are essential, the mechanism of stroke is often related to vasculitis complicated by arterial stenosis and occlusion. Its recurrence is often attributed to early steroid resistance or late GCA relapse, so immunosuppressive treatment is often reinforced. However, many questions concerning the mechanisms of stroke remain elusive, and no review to date has examined the whole data set concerning GCA-related stroke. We therefore undertook this scoping review. GCA-related stroke does not necessarily display general signs and inflammatory parameters are sometimes normal, so clinicians should observe caution. Ischemic lesions often show patterns predating watershed areas and are associated with stenosis or thrombosis of the respective arteries, which are often bilateral. Lesions predominate in the siphon in the internal carotid arteries, whereas all the vertebral arteries may be involved with a predominance in the V3-V4 segments. Ultrasonography of the cervical arteries may reveal edema of the intima (halo sign), which is highly sensitive and specific of GCA, and precedes stenosis. The brain arteries are spared although very proximal arteritis may rarely occur, if the patient has microstructural anatomical variants. Temporal artery biopsy reveals the combination of mechanisms leading to slit-like stenosis, which involves granulomatous inflammation and intimal hyperplasia. The lumen is sometimes occluded by thrombi (<15%), suggesting that embolic lesions may also occur, although imaging studies have not provided strong evidence for this. Moreover, persistence of intimal hyperplasia might explain persisting arterial stenosis, which may account for delayed stroke occurring in watershed areas. Other possible mechanisms of stroke are also discussed. Overall, GCA-related stroke mainly involves hemodynamic mechanisms. Besides early diagnosis and treatment initiation, future studies could seek to establish specific preventive or curative treatments using angioplasty or targeting intimal proliferation.

Giant cell arteritis-related cerebrovascular ischemic events: a French retrospective study of 271 patients, systematic review of the literature and meta-analysis

BackgroundCerebrovascular ischemic events (CIE) are among the most severe complications of giant cell arteritis (GCA). Heterogeneity between different studies in the definition of GCA-related CIE leads to uncertainty regarding their real prevalence. The aim of our study was to evaluate the prevalence and describe the characteristics of GCA-related CIE in a well-phenotyped cohort completed by a meta-analysis of the existing literature.MethodsIn this retrospective study performed in the Lille University Hospital, all consecutive patients with GCA according to American College of Rheumatology (ACR) diagnostic criteria were included from January 1, 2010, to December 31, 2020. A systematic review of the literature using MEDLINE and EMBASE was performed. Cohort studies of unselected GCA patients reporting CIE were included in the meta-analysis. We calculated the pooled summary estimate of GCA-related CIE prevalence.ResultsA total of 271 GCA patients (89 males, mean age 72 ± 9 years) were included in the study. Among them, 14 (5.2%) presented with GCA-related CIE including 8 in the vertebrobasilar territory, 5 in the carotid territory, and 1 patient having multifocal ischemic and hemorrhagic strokes related to intra-cranial vasculitis. Fourteen studies were included in the meta-analysis, representing a total population of 3553 patients. The pooled prevalence of GCA-related CIE was 4% (95% CI 3–6, I2 = 68%).Lower body mass index (BMI), vertebral artery thrombosis on Doppler US (17% vs 0.8%, p = 0.012), vertebral arteries involvement (50% vs 3.4%, p < 0.001) and intracranial arteries involvement (50% vs 1.8%, p < 0.001) on computed tomography angiography (CTA) and/or magnetic resonance angiography (MRA), and axillary arteries involvement on positron emission computed tomography (PET/CT) (55% vs 20%, p = 0.016) were more frequent in GCA patients with CIE in our population.ConclusionsThe pooled prevalence of GCA-related CIE was 4%. Our cohort identified an association between GCA-related CIE, lower BMI, and vertebral, intracranial, and axillary arteries involvement on various imaging modalities.

Atteintes coronariennes et artérite a cellules géantes : à propos de 2 cas et revue de la littérature

IntroductionCoronaritis is a rare but serious complication of giant-cell arteritis (GCA), with an estimated prevalence of less than 1%, however difficult to establish, and of early onset. MethodsWe describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication. ResultsThe first patient presented with stable angina on common trunk coronaritis with ostial stenosis. Corticosteroid combined with tocilizumab from the outset resulted in improvement. Angioplasty was performed at 6months with good outcome. The second patient presented with asymptomatic tritruncular ostial coronaritis. Corticosteroid allowed clinic-biological improvement of GCA. Two years later, he presented relapse with an acute coronary syndrome, with favorable evolution after angioplasty, increase of corticosteroids and addition of tocilizumab. ConclusionPatients presented were successfully treated with corticosteroids combined with tocilizumab and angioplasty of their coronary stenoses. Efficacy of tocilizumab in GCA has not been evaluated especially on coronaritis due to the rarity of this complication. Our experience and the cases reported in the literature suggest good results of angioplasty in this indication. Studies with long-term follow-up will be necessary to evaluate the risk of restenosis.

A severe cerebral infarction associated with giant cell arteritis, which developed during tocilizumab therapy and was successfully treated with intravenous cyclophosphamide.

Giant cell arteritis (GCA) is a large vessel vasculitis that primarily involves aorta and its major branches. Cerebral infarction is a serious complication that can occur secondary to GCA in up to 3% of patients with a mortality rate of over 50%. Due to the rarity of this severe complication, no therapeutic strategies are currently available. Furthermore, despite the recent progress in molecular-targeted therapy for GCA, it remains unknown whether tocilizumab is effective for severe ischemic complications such as cerebral infarction. The accumulation of individual cases in which this fatal complication could be treated is apparently required to build a better management of the disease. We present our case of GCA that developed severe cerebral infarction during high-dose glucocorticoid and tocilizumab therapy, and its symptoms and image findings were improved by switching to intravenous cyclophosphamide. Our case suggests that an intensive immunosuppressive therapy, including cyclophosphamide, may be necessary to stabilise this fatal complication of GCA.

Neurological complications of giant cell arteritis: A study of 15 cases and a review of the literature

Giant cell arteritis (GCA) is considered a neuro-ophthalmologic emergency. New-onset headache in patients aged 50 years and above with elevated erythrocyte sedimentation rate should prompt evaluation for GCA. Retrospective study of 15 patients presenting with GCA from 1991 to 2008 at the Mohamed V Military hospital at Rabat and Avicenne Military hospital of Marrakech. Fifteen cases were recorded, with female predominance (male to female ratio 2:3) and a mean age of 63 years (range: 55-83 years). All patients (100%) presented with headache. The headache was isolated in 20% of cases and neuro-ophthalmic complications were found in 73% of cases. Biopsy was conclusive for GCA in 67% of cases and all of our patients were placed on steroids with spectacular improvement. New-onset headache in patients aged 50 years and above should prompt evaluation for GCA. Steroids, especially during the acute phase, must be started urgently to avoid irreversible neurological impairment.

Intraorbital findings in giant cell arteritis on black blood MRI

ObjectiveBlindness 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.MethodsIn 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.ResultsEighteen 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.ConclusionsBB-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.

Evidence-based Role of Aspirin in Giant Cell Arteritis: A Literature Review.

Giant cell arteritis (GCA), or temporal arteritis, is a medium to large vessel vasculitis seen in the elderly. Its presentation varies from fever of unknown origin to cranial ischemic complications including complete vision loss. The early initiation of steroids is key to preventing complications of GCA. Here we discuss the role of aspirin in the treatment of GCA, both as an antithrombotic agent and its increasingly utilized anti-inflammatory properties. The aim of this review article is to examine the evidence behind the use of aspirin as an adjunct to steroids for the prevention of cranial ischemic complications.

Magnetic resonance imaging-based diagnosis of aortitis preceding development of a thoracic aneurysm in a patient with giant cell arteritis: a case report

BackgroundInflammatory manifestation in the aortic arch can be a complication of giant cell arteritis (GCA), potentially requiring surgical therapy in the case of aneurysmatic dilatation.Case summaryWe report the case of a 73-year-old female patient with GCA in whom a typical appearance of arteritis was visualized on magnetic resonance imaging of the superficial temporal arteries. Additionally, ectasia (4.7 cm) of the ascending aorta with a mural rim of increased contrast media uptake was detected at the time of the initial diagnosis, which is an indicator of aortitis. While the diameter had only minimally increased in a computed tomography angiography (CTA) examination after 8 months, a subsequent CTA revealed an increased diameter of 5.8 cm and maximum at the level of the ascending aorta another 22 months later, indicating urgent surgery to replace the ascending aorta.DiscussionMagnetic resonance imaging can detect silent, generalized manifestations of GCA such as severe aortitis, which may possibly lead to aneurysmatic dilatation, urging closer follow-up imaging. Detection of the ongoing process and subsequent follow-up imaging protects patients by avoiding rupture.

Ocular Complications of Giant Cell Arteritis: An Acute Therapeutic Emergency.

The risk of blindness, due to acute ischemic ocular events, is the most feared complication of giant cell arteritis (GCA) since the middle of the 20th century. A decrease of its rate has occurred after the advent of corticoid therapy for this vasculitis, but it seems to have stabilized since then. Early diagnosis and treatment of GCA is key to reducing its ocular morbidity. However, it is not uncommon for ophthalmological manifestations to inaugurate the disease, and the biological inflammatory reaction may be mild, making its diagnosis more challenging. In recent years, vascular imaging has opened up new possibilities for the rapid diagnosis of GCA, and ultrasound has taken a central place in fast-track diagnostic processes. Corticosteroid therapy remains the cornerstone of treatment and must begin immediately in patients with visual symptoms and suspicion of GCA. In that situation, the administration route of corticotherapy, intravenous or oral, is less important than its speed of delivery, any hour of delay worsening the prognosis.

Neurologic manifestations of giant cell arteritis.

Giant cell arteritis, the most frequent form of vasculitis in persons over 50years of age, is a granulomatous chronic vasculitis involving large and medium-sized vessels, most commonly the temporal and other cranial arteries. This common, treatable condition is associated with various clinical symptoms, including neurological ones, affecting both the central and peripheral nervous systems. In this review, we discuss the cranial and extra cranial neurological complications of giant cell arteritis, to help avoid the many pitfalls in the diagnosis of giant cell arteritis.

Tocilizumab in visual involvement of giant cell arteritis: a multicenter study of 471 patients.

Background:Visual involvement is the most feared complication of giant cell arteritis (GCA). Information on the efficacy of tocilizumab (TCZ) for this complication is scarce and controversial.Objective:We assessed a wide series of GCA treated with TCZ, to evaluate its role in the prevention of new visual complications and its efficacy when this manifestation was already present before the initiation of TCZ.Design:This is an observational multicenter study of patients with GCA treated with TCZ.Methods:Patients were divided into two subgroups according to the presence or absence of visual involvement before TCZ onset. Visual manifestations were classified into the following categories: transient visual loss (TVL), permanent visual loss (PVL), diplopia, and blurred vision.Results:Four hundred seventy-one GCA patients (mean age, 74 ± 9 years) were treated with TCZ. Visual manifestations were observed in 122 cases (26%), of which 81 were present at TCZ onset: PVL (n = 60; unilateral/bilateral: 48/12), TVL (n = 17; unilateral/bilateral: 11/6), diplopia (n = 2), and blurred vision (n = 2). None of the patients without previous visual involvement or with TVL had new episodes after initiation of TCZ, while only 11 out of 60 (18%) patients with PVL experienced some improvement. The two patients with diplopia and one of the two patients with blurred vision improved.Conclusion:TCZ may have a protective effect against the development of visual complications or new episodes of TVL in GCA. However, once PVL was established, only a few patients improved.

Carotid Artery Stenosis and Ischemic Strokes in Patients with Giant Cell Arteritis: A Characteristic Pattern-Literature Review and Case Report.

Purpose Ischemic stroke is a relatively rare complication of giant cell arteritis often accompanied by vessel stenosis. Our purpose was to compare the location of internal carotid artery stenosis in GCA patients by performing a literature review suggesting a specific and characteristic pattern. Methods We performed a PubMed research including all articles and cited articles reporting cases and case series about giant cell arteritis patients with internal carotid artery stenosis and ischemic strokes. Results In this case series 39 cases were included. We found a clear tendency of giant cell arteritis-related stenosis to be in the intracranial segments (35/39 (89.7%)). Only in 8/39 (20.5%) patients there was further involvement of extracranial segments. Many cases (27/39 [69.2%]) showed a bilateral involvement. Discussion This literature review reveals a specific pattern of internal carotid artery involvement in patients with giant cell arteritis and ischemic strokes. To our knowledge this pattern has not been reported as a sign strongly pointing toward giant cell arteritis before. We have not found case reports mentioning other common types of vasculitis reporting this involvement pattern. Conclusion Internal carotid artery stenosis and ischemic stroke is a rare complication in patients with giant cell arteritis. Considering the characteristic features of bilateral distal internal carotid artery stenosis giant cell arteritis should be suspected which potentially leads to an early diagnosis and immunotherapy.

159 Acute coronary syndrome in patient with giant cell arteritis: a fascinating case

Abstract Aims The diagnosis of acute coronary syndrome with persistent ST segment elevation, although apparently simple to detect by electrocardiographic abnormalities, can sometimes be insidious due to a difficult differential diagnosis with aortic dissection. Performing a coronary angiography in case of aortic dissection can be potentially life-threatening so, when suspected, this diagnosis needs to be rule out. Furthermore, coronary vasculitis is a rare but devastating complication of giant cell arteritis. Methods and results We describe a case of 62-year-old man hospitalized for acute coronary syndrome with persistent ST segment elevation involving the inferior wall. Ha was a heavy smoker with familiar history of cardiovascular disease and recent suspicion of Horton arteritis with aortic involvement under investigation, no previous cardiological events. Presenting symptoms are chest pain, sweating, bradycardia, and hypotension. In the emergency department, due to the history of suspected arteritis, chest CT scan was performed in order to rule out the hypothesis of aortic dissection before proceeding to coronarography. No signs of aortic rupture but the presence of thickening of the aorta from the arch to the carrefour was found. The coronary angiography showed critical stenosis in the medial tract of the circumflex artery and thrombotic subocclusion in the middle segment of the right coronary (culprit lesion—Figure 1) that was treated with PCI using drug-eluting stent. The echocardiogram showed mild left ventricular disfunction (EF 53%) due to posterolateral hypokinesia, no other pathological findings. Blood tests revealed anaemia, elevated platelets, and an increase of inflammation markers. Peak Hs-troponin was 18 000 ng/L (n.v. &amp;lt; 0.02 ng/ml). After 2 days we performed elective revascularization of the circumflex artery using a drug-eluting stent. Considering the medical history, we performed temporal artery biopsy and a total body PET scan that showed accumulation of radioactive tracer from the aortic arch to the sub-renal abdominal aorta, with a thickened wall. After that empirical corticosteroid therapy was started and some days later the result of the temporal artery biopsy confirmed the diagnosis of Horton arteritis. The patient was discharged to a rehabilitation structure in good general condition with cardioactive and corticosteroid therapy after 9 days of hospitalization. Conclusions Horton arteritis usually involves the external carotid artery and its branches, but sometimes inflammation of the aorta occurs in a subgroup of patients and rarely can involve the coronary arteries. An acute coronary syndrome in patients with Horton arteritis can be very insidious because aortic dissection can be the hidden cause, so chest CT scan must be performed before coronary angiography to avoid acute complication associated with an elevated mortality. Furthermore, being the vasculitic inflammation the trigger of the coronary involvement immunosuppressive therapy could be helpful to prevent subsequent acute coronary events.

Ultrasound Technologies and the Diagnosis of Giant Cell Arteritis.

Giant cell arteritis (GCA) is a primary autoimmune vasculitis that specifically affects medium-sized extracranial arteries, like superficial temporal arteries (TAs). The most important data to be considered for the ultrasound (US) diagnosis of temporal arteritis are stenosis, acute occlusions and “dark halo” sign, which represent the edema of the vascular wall. The vessel wall thickening of large vessels in GCA can be recognized by the US, which has high sensitivity and is facile to use. Ocular complications of GCA are common and consist especially of anterior arterial ischemic optic neuropathies or central retinal artery occlusion with sudden, painless, and sharp loss of vision in the affected eye. Color Doppler imaging of the orbital vessels (showing low-end diastolic velocities and a high resistance index) is essential to quickly differentiate the mechanism of ocular involvement (arteritic versus non-arteritic), since the characteristics of TAs on US do not correspond with ocular involvement on GCA. GCA should be cured immediately with systemic corticosteroids to avoid further visual loss of the eyes.

Atypical Horton's arteritis with oculomotor nerves involvement and acute brain ischemic lesion: A case report

Background: Oculomotor nerves palsy is a less common complication of giant cell arteritis (GCA) than ischemic optic neuritis, nevertheless it occurs in at least 10% of cases. Other rare complications are cerebrovascular ischemic events, which occur in about 7% of cases. The latter can underlie intra-extracranial vessel stenosis or occlusion, mainly in vertebro-basilar territory, but also an arterial-embolic stroke is possible. Aims: We first report a case of histologically proven GCA in which occurred two rare clinical manifestations, both peripheral oculomotor nerves palsy and acute cerebral ischemic stroke. Besides, PET-MRI compatible with aortitis was found, and arterial-embolic etiology of the stroke was hypothesized.

Vascular endothelial growth factor haplotypes are associated with severe ischaemic complications in giant cell arteritis regardless of the disease phenotype.

To determine whether functional vascular endothelial growth factor (VEGF) polymorphisms influence the expression of the clinical phenotype of giant cell arteritis (GCA). We also evaluated whether VEGF polymorphism is associated with the development of severe ischaemic manifestations in patients with GCA regardless of the clinical phenotype, classic cranial GCA or predominantly extracranial GCA large vessel vasculitis (LVV). VEGF rs833061 T/C, rs2010963 G/C and rs3025039 C/T polymorphisms were genotyped in 185 patients with biopsy-proven cranial GCA, 105 with extracranial LVV-GCA and 490 healthy controls. Allelic combinations (haplotypes) of VEGF were carried out. Comparisons were performed between patients with GCA and healthy controls as well as between patients with GCA stratified according to the clinical phenotype and the presence of severe ischaemic manifestations. No significant differences in genotype, allele, and haplotype frequencies of VEGF were found between patients with GCA and healthy controls as well as between GCA patients with the classic cranial pattern and the extracranial LVV-GCA pattern of the disease. However, the VEGF CGC haplotype (OR= 1.63 [1.05-2.53]) and the CGT haplotype (OR= 2.55 [1.10-5.91]) were significantly more frequent in GCA patients with severe ischaemic complications compared to those patients without these complications. VEGF haplotypes seem to play a role in the development of severe ischaemic manifestations in GCA patients, regardless of the clinical phenotype of expression of the disease.