scholarly journals The Thromboembolic Risk in Giant Cell Arteritis: A Critical Review of the Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
A. Guida ◽  
A. Tufano ◽  
P. Perna ◽  
P. Moscato ◽  
M. T. De Donato ◽  
...  

Giant cell arteritis is a systemic vasculitis characterized by granulomatous inflammation of the aorta and its main vessels. Cardiovascular risk, both for arterial and venous thromboembolism, is increased in these patients, but the role of thromboprophylaxis is still debated. It should be suspected in elderly patients suffering from sudden onset severe headaches, jaw claudication, and visual disease. Early diagnosis is necessary because prognosis depends on the timeliness of treatment: this kind of arteritis can be complicated by vision loss and cerebrovascular strokes. Corticosteroids remain the cornerstone of the pharmacological treatment of GCA. Aspirin seems to be effective in cardiovascular prevention, while the use of anticoagulant therapy is controversial. Association with other rheumatological disease, particularly with polymyalgia rheumatica is well known, while possible association with antiphospholipid syndrome is not established. Large future trials may provide information about the optimal therapy. Other approaches with new drugs, such as TNF-alpha blockades, Il-6 and IL-1 blockade agents, need to be tested in larger trials.

2019 ◽  
Vol 11 ◽  
pp. 1759720X1982722 ◽  
Author(s):  
Candice Low ◽  
Richard Conway

Giant cell arteritis (GCA) is the most common form of systemic vasculitis. It is a potentially severe disease with 25% of patients suffering vision loss or stroke. Our treatment paradigm is based on glucocorticoids. Glucocorticoids are required in high doses for prolonged periods and subsequently are associated with a significant amount of treatment-related morbidity. Alternative treatment options are urgently needed to minimize these glucocorticoid adverse events. Many other agents, such as methotrexate and tumour necrosis factor alpha inhibitors have been used in GCA, with limited or no evidence of benefit. Our emerging understanding of the pathogenic processes involved in GCA has led to an increased interest in the use of biologic agents to treat the disease. Two randomized controlled trials have recently reported dramatic effects of the use of the interleukin-6 targeted biologic tocilizumab in GCA, with significant increases in remission rates and decreases in glucocorticoid burden. While encouraging, longer-term and additional outcomes are awaited to clarify the exact positioning of tocilizumab in the treatment approach. Emerging data for other biologic agents, particularly abatacept and ustekinumab, are also encouraging but less well advanced. We are at the dawn of a new era in GCA treatment, but uncertainties and opportunities abound.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1540.2-1540
Author(s):  
B. Ince ◽  
S. Artan ◽  
Y. Yalçinkaya ◽  
B. Artim-Esen ◽  
A. Gül ◽  
...  

Background:Development of organ damage is a major concern in patients with systemic vasculitis. Treatment may also contribute to this important outcome. Scoring systems has been developed to evaluate organ damage in systemic vasculitis and specifically for large vessel vasculitis (1).Objectives:We aimed to investigate permanent organ damage and determining factors in our giant cell arteritis GCA cohort.Methods:Organ damage detected at the time of diagnosis and / or follow-up and irreversible for at least 3 months in GCA patients followed up between 1998-2018 were recorded by using Vasculitis Damage Index (VDI) and Vascular Vasculitis Damage Index (LVVID) fom patient records of our vasculitis clinic. In the statistical evaluation, chi-square, students t-test and logistic regression analysis were used.Results:Eighty-nine patients (64% women, mean age 67.9 ± 9.1) included in the study, the mean follow-up duration was 61.6 ± 58.6 months. All organ damage findings according to both VDI and LVVID are shown in table-1. In this cohort, cardiovascular damage items and diabetes mellitus were prevalent at baseline. At least one damage item was present in 53 (59,5%) according to VDI; 54 (%60,7) according to LVVID and agreement was high between two damage indices (kappa=0.97). Forty-seven of patients (52%) had a damage item presumably with contribution of GC treatment e.g. locomotor system findings, hypertension, diabetes and cataract; 12 (13,5%) had damage items related to disease (total or partial vision loss, ischemic optical neuropathy). Mean time to diagnosis after initial symptoms was longer in patients with permanent vision loss (10,2±4,3 vs. 5,2±1,2 months p=0.006). The presence of damage was associated with flares in univariate and multivariate analysis (29/54 vs. 2/35 p<0,001 OR=19 %95 GA 4,2– 87,9). All patients who had a flare during the first year (n = 15) developed signs of damage at follow-up. No association was found between the development of organ damage and the age of diagnosis, the time between first complaint and diagnosis, presence of cranial, ophthalmologic findings, PET-CT positivity, cumulative steroid dose, and DMARD use.Conclusion:In our study, permanent organ damage was analysed by using diffrerent indices. In this patient population baseline cardiovascular damage and diabetes mellitus were frequent as expected but information for osteoporosis was lacking. More than half of the patients had damage and significant part of the present items was considered due to corticosteroid treatment. The most common damage item developed was osteoporosis. There was a very good agreement between the two indices, despite few specific items in LVVID. The striking relationship of disease flare with damage and frequency of visual problems despite treatment indicate the necessity of new treatment strategies.References:[1]Kermani, T.A., et al.,Evaluation of damage in giant cell arteritis.Rheumatology (Oxford), 2018.57(2): p. 322-328.Disclosure of Interests: :None declared


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Jose R. Zaragoza ◽  
Natalia Vernon ◽  
Gisoo Ghaffari

Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries that mainly affects the external carotid artery. It is a diagnosis of the elderly that typically presents as low-grade fever, temporal tenderness, claudication of the jaw, and in some patients vision loss. In cases where GCA presents with atypical manifestations, the diagnosis may be more difficult, causing a delay in both diagnosis and treatment and ultimately leading to irreversible complications. In this paper, we present an atypical presentation of GCA with symptoms of neck swelling and lingual pain in an elderly female. These symptoms progressed to bilateral necrosis and eventual dislodgement of the tongue. Lingual necrosis is a severe potential complication in GCA. In patients presenting with lingual swelling, pain, and discoloration, GCA should be suspected and prompt therapy should be initiated to avoid irreversible complications.


2016 ◽  
Vol 20 (90) ◽  
pp. 1-238 ◽  
Author(s):  
Raashid Luqmani ◽  
Ellen Lee ◽  
Surjeet Singh ◽  
Mike Gillett ◽  
Wolfgang A Schmidt ◽  
...  

Background Giant cell arteritis (GCA) is a relatively common form of primary systemic vasculitis, which, if left untreated, can lead to permanent sight loss. We compared ultrasound as an alternative diagnostic test with temporal artery biopsy, which may be negative in 9–61% of true cases. Objective To compare the clinical effectiveness and cost-effectiveness of ultrasound with biopsy in diagnosing patients with suspected GCA. Design Prospective multicentre cohort study. Setting Secondary care. Participants A total of 381 patients referred with newly suspected GCA. Main outcome measures Sensitivity, specificity and cost-effectiveness of ultrasound compared with biopsy or ultrasound combined with biopsy for diagnosing GCA and interobserver reliability in interpreting scan or biopsy findings. Results We developed and implemented an ultrasound training programme for diagnosing suspected GCA. We recruited 430 patients with suspected GCA. We analysed 381 patients who underwent both ultrasound and biopsy within 10 days of starting treatment for suspected GCA and who attended a follow-up assessment (median age 71.1 years; 72% female). The sensitivity of biopsy was 39% [95% confidence interval (CI) 33% to 46%], which was significantly lower than previously reported and inferior to ultrasound (54%, 95% CI 48% to 60%); the specificity of biopsy (100%, 95% CI 97% to 100%) was superior to ultrasound (81%, 95% CI 73% to 88%). If we scanned all suspected patients and performed biopsies only on negative cases, sensitivity increased to 65% and specificity was maintained at 81%, reducing the need for biopsies by 43%. Strategies combining clinical judgement (clinician’s assessment at 2 weeks) with the tests showed sensitivity and specificity of 91% and 81%, respectively, for biopsy and 93% and 77%, respectively, for ultrasound; cost-effectiveness (incremental net monetary benefit) was £485 per patient in favour of ultrasound with both cost savings and a small health gain. Inter-rater analysis revealed moderate agreement among sonographers (intraclass correlation coefficient 0.61, 95% CI 0.48 to 0.75), similar to pathologists (0.62, 95% CI 0.49 to 0.76). Limitations There is no independent gold standard diagnosis for GCA. The reference diagnosis used to determine accuracy was based on classification criteria for GCA that include clinical features at presentation and biopsy results. Conclusion We have demonstrated the feasibility of providing training in ultrasound for the diagnosis of GCA. Our results indicate better sensitivity but poorer specificity of ultrasound compared with biopsy and suggest some scope for reducing the role of biopsy. The moderate interobserver agreement for both ultrasound and biopsy indicates scope for improving assessment and reporting of test results and challenges the assumption that a positive biopsy always represents GCA. Future work Further research should address the issue of an independent reference diagnosis, standards for interpreting and reporting test results and the evaluation of ultrasound training, and should also explore the acceptability of these new diagnostic strategies in GCA. Funding The National Institute for Health Research Health Technology Assessment programme.


2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Nadia Ahmad ◽  
Elizabeth Price ◽  
Areli Cuevas-Ocampo ◽  
Khin Yein ◽  
Azeem Ahmed

Abstract Introduction This intriguing case describes a patient in who initial giant cell arteritis (GCA)/temporal arteritis (TA) presentation was preceded by bilateral acute anterior uveitis. He presented several months later after being treated for GCA with new neurological symptoms not typical of ischaemic cerebrovascular accident (CVA) on brain imaging. After ruling out a variety of differentials including an infection, he was treated for cerebral vasculitis secondary to temporal arteritis confirmed on brain biopsy which remains gold standard for diagnosis. Case description A 73-year-old patient with a background history of hypertension and mild asthma presented with three week history of ocular pain, headache and photosensitivity after a fall. CT head and lumbar puncture (LP) were unremarkable. He was diagnosed with bilateral acute anterior uveitis by ophthalmologists and treated with topical cyclopentolate and dexamethasone . In view of headaches, scalp tenderness, jaw claudication and raised inflammatory markers he was treated with 60mg of prednisolone for presumed giant cell arteritis (GCA) and temporal artery biopsy (TAB) was organised. He showed marked symptomatic improvement on steroids. Inflammatory markers normalised (erythrocyte sedimentation rate (ESR) 77 → 5 and C-reactive protein (CRP) 130 → <1). Temporal artery biopsy was negative, but took more than four weeks after starting steroids and was only 9mm in length. Serum screening was unremarkable for complements C3,4, antinuclear antibodies (ANA), anti neutrophil cytoplasmic antibodies (ANCA), bacterial or viral antibodies. Ten months later he was admitted with a two-week history of gradually worsening bilateral lower limb weakness on the background of chronic lower back pain. Magnetic resonance imaging (MRI) head showed parasagittal abnormalities which were thought to be atypical for ischemic infarction. Intracranial angiogram did not reveal any pathology. LP demonstrated elevated white cells (18 × 106/L – normal <5 × 106/L) and protein 0.61g/L (normal < 0.15-0.45g/L) with negative oligoclonal bands. The serology for neuronal autoantibodies and quantiferon was negative. ESR was elevated (50). Echocardiogram showed no vegetations. He was managed for acute cerebral vasculitis with methylprednisolone and pulsed cyclophosphamide (CYC). He also underwent a repeat TAB which was normal. In view of clinical deterioration he underwent repeat MRI head and spine which showed persistent active inflammation. Brain biopsy was organised which confirmed granulomatous inflammation with multinucleated giant cells. Unfortunately he continued to deteriorate, suffered from multiple infections and sadly passed away at his home with his family. Discussion Giant cell arteritis is a systemic vasculitis characterized by granulomatous inflammation of aorta and its main vessels. Visual complications are mostly due to vasculitis of posterior ciliary arteries. Uveitis as a presenting feature of GCA is uncommon. We should be aware that, although unusual, uveitis in elderly patients can be a presenting feature of GCA. Cardiovascular risk is increased in these patients. Several case series of myocardial infarction and stroke have been reported. About 30% of patients present with neurological manifestations, the most common are neuropathies (14%), including mono- and polyneuropathies of the limbs; stroke has been extensively described (5-20%), particularly vertebrobasilar ischemia. Cerebral vasculitis may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation of systemic vasculitis. In GCA, the involvement of CNS arteries is very rare (<2%). Our patient’s imaging revealed bilateral parafalcine frontal lobe changes in anterior cerebral artery territory. However, infarction in this territorial area is quite rare unless there is space occupying lesion or anatomical anomalies of vasculature. In our patient the MRI appearances were not convincing for ischaemic infarction. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on a combination of clinical, laboratory and imaging findings. In systemic vasculitis an acute inflammatory response with raised ESR and CRP may be present. CSF studies reveal mild lymphomonocytic pleocytosis or protein elevation in more than 90%. Magnetic resonance imaging, with or without contrast, is the investigation of choice to detect and monitor cerebral involvement. The treatment recommendations are derived from protocols for systemic vasculitides. A combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. Methotrexate, azathioprine and mycophenolate mofetil can be used for maintenance therapy similar to ANCA associated vasculitis. Key learning points Our case highlighted the rare presenting feature of GCA in the form of bilateral uveitis. Our patient was at high risk for developing ischaemic cerebral vascular event in view of large vessel vasculitis, his age and co-morbid hypertension but radiological imaging wasn’t typical for this and raised the suspicion of active cerebral vasculitis.  One should suspect multifocal brain disease like vasculitis when neurological deficit can’t be explained easily by territorial distribution of cerebral circulation. Cerebral vasculitis can be suspected on brain imaging and confirmed with biopsy. It is important to make this diagnosis as the treatment is immunosuppression different from that of a typical stroke and can be rewarding. Our patient was managed with immunosuppressive therapy but continued to deteriorate that prompted the need for brain biopsy which remains the gold standard for diagnosing cerebral vasculitis. Conflicts of interest The authors have declared no conflicts of interest.


2019 ◽  
Vol 16 (1) ◽  
pp. 109-114
Author(s):  
V. A. Chernukha ◽  
D. S. Atarschikov ◽  
N. V. Khamnagdaeva ◽  
I. V. Pozharov

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.


Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1683
Author(s):  
Milagros Mateos-Olivares ◽  
Luis García-Onrubia ◽  
Fco. Javier Valentín-Bravo ◽  
Rogelio González-Sarmiento ◽  
Maribel Lopez-Galvez ◽  
...  

Diabetic macular oedema (DMO) is one of the leading causes of vision loss associated with diabetic retinopathy (DR). New insights in managing this condition have changed the paradigm in its treatment, with intravitreal injections of antivascular endothelial growth factor (anti-VEGF) having become the standard therapy for DMO worldwide. However, there is no single standard therapy for all patients DMO refractory to anti-VEGF treatment; thus, further investigation is still needed. The key obstacles in developing suitable therapeutics for refractory DMO lie in its complex pathophysiology; therefore, there is an opportunity for further improvements in the progress and applications of new drugs. Previous studies have indicated that Rho-associated kinase (Rho-kinase/ROCK) is an essential molecule in the pathogenesis of DMO. This is why the Rho/ROCK signalling pathway has been proposed as a possible target for new treatments. The present review focuses on the recent progress on the possible role of ROCK and its therapeutic potential in DMO. A systematic literature search was performed, covering the years 1991 to 2021, using the following keywords: “rho-Associated Kinas-es”, “Diabetic Retinopathy”, “Macular Edema”, “Ripasudil”, “Fasudil” and “Netarsudil”. Better insight into the pathological role of Rho-kinase/ROCK may lead to the development of new strategies for refractory DMO treatment and prevention.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 689.1-690
Author(s):  
S. Monti ◽  
L. Dagna ◽  
C. Campochiaro ◽  
A. Tomelleri ◽  
G. Zanframundo ◽  
...  

Background:Giant cell arteritis (GCA) is the most frequent systemic vasculitis after the age of 50 years old. Recent interest in the processes of immune and vascular aging have been proposed as a disease risk factor. Data on the impact of age at diagnosis of GCA on the clinical course of the disease are scarceObjectives:To assess the role of age at diagnosis of GCA on the risk and time to relapseMethods:Centres participating in the Italian Society of Rheumatology Vasculitis Study Group retrospectively enrolled patients with a diagnosis of GCA until December 2019. The cohort was divided in tertiles according to age at diagnosis (≤ 72; 73-79; > 79 years old). Negative binomial regression was used to assess the relapse rate according to age groups, and Cox regression for time to first relapse.Results:Of 720 patients enrolled in 14 Italian reference centres, 711 had complete follow-up data (female 50%; mean age 75±7). Median follow-up duration was 34 months (IQR 16;70). Patients in the older group at diagnosis (> 79 years) had more frequent visual loss compared to the 73-79 and ≤ 72 age groups (31% vs 20% vs 7%; p<0.001), but lower rates of general symptoms (56% vs 70% vs 77%; p<0.001). Large-vessel (LV)-GCA was less frequent in the older group (18% vs 22% vs 43%; p<0.001). At least one relapse occurred in 47% of patients. Median time to relapse was 12 months (IQR 6;23). Age did not influence the rate of relapses [18 per 100 persons/years (95%CI 15;21) vs 19 (95% CI 17;22) vs 19 (95%CI 17;22)], nor the time to first relapse (Figure 1). LV-GCA, presentation with significantly elevated c-reactive protein (> 50 mg/L) and general symptoms were independent predictors of relapse.Conclusion:Age at diagnosis of GCA influenced the clinical presentation and risk of ischaemic complications, but did not affect the relapse rate during follow-up. LV-GCA occurred more frequently in younger patients and was an independent predictor of relapse risk, highlighting the need for a correct characterization of the clinical subtype at the early stages of disease.Disclosure of Interests:Sara Monti: None declared, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Alessandro Tomelleri: None declared, Giovanni Zanframundo: None declared, Catherine Klersy: None declared, Francesco Muratore: None declared, Luigi Boiardi: None declared, Roberto Padoan: None declared, Mara Felicetti: None declared, Franco Schiavon: None declared, Milena Bond: None declared, Alvise Berti: None declared, Roberto Bortolotti: None declared, Carlotta Nannini: None declared, Fabrizio Cantini: None declared, Alessandro Giollo: None declared, Edoardo Conticini: None declared, angelica gattamelata: None declared, Roberta Priori: None declared, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer, Elena Treppo: None declared, Giacomo Emmi: None declared, Martina Finocchi: None declared, Giulia Cassone: None declared, Ariela Hoxha Speakers bureau: Celgene, UCB, Novartis, Sanofi, Werfen, Rosario Foti Consultant of: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Speakers bureau: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Michele Colaci: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Carlo Salvarani: None declared, Carlomaurizio Montecucco: None declared


Author(s):  
CG Davies ◽  
DJ May

A knowledge of the disease process of giant cell arteritis and its diagnosis can help a surgeon to decide which patients will benefit from a biopsy being performed and identify where a biopsy would be of no value in their management. This article discusses the issues involved.


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