scholarly journals Arteritis temporalis

2009 ◽  
Vol 18 (1) ◽  
Author(s):  
Øyvind Palm

Giant cell arteritis (GCA) is a common systemic vasculitis in elderly Norwegian patients. Scandinavian studies confirm that GCA usually starts rather acute and is diagnosed within less than two months in most cases. Temporal headache, tenderness and reduced pulsation of the temporal artery combined with significantly elevated ESR are typical features of the disease. A declining incidence from North to South across Europe has been found. The estimated annual incidence in Norway is 20.6-29.1 per 100.000 persons aged 50 years or more, which is among the highest worldwide. The high incidence in the Northern countries and the still incomplete understood ethiopathogenesis should encourage Scandinavian research on GCA.

2018 ◽  
Vol 15 (6) ◽  
pp. 51-58
Author(s):  
Adina Cociorvei ◽  
Mădălina Ababei

AbstractGiant cell arteritis (GCA), or temporal arteritis, is the most common systemic vasculitis, and the greatest risk factor for developing GCA is aging. The disease almost never occurs before age 50, and its incidence rises steadily thereafter, peaking between ages 70 to 79, the risk of development being two times higher in women.Polymialgia rheumatica (PMR) is an inflammatory rheumatic condition characterized clinically by aching and morning stiffness at the shoulders, hip girdle, and neck. PMR is almost exclusively a disease of adults over the age of 50, with a prevalence that increases progressively with advancing age. The peak incidence of PMR occurs between ages 70 and 80, the same as in the case ofGCA. PMRis 2-3 times more common in women than in men.PMR is two to three times more common than GCA and occurs in about 50% of patients with GCA. The percentage of patients with PMR who experience GCA at some point varies widely in reported series ranging from 5 to 30 percent. PMR can precede, accompany or follow GCA. The diagnostic in the case of PMR is made first of all on clinical features, in the patients in whom another disease to explain the findings is not present. For GCA we must follow the diagnostic algorithm presented below (figure 1) and keep in mind that a negative result for temporal artery biopsy does not exclude the diagnostic if clinical suspicion of GCA is highWe present the case of a 81 year-old male with signs and symptoms from both conditions, PMR and GCA.


2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Alfredomaria Lurati ◽  
Luca Bertani ◽  
Katia Angela Re ◽  
Mariagrazia Marrazza ◽  
Daniela Bompane ◽  
...  

Giant cell arteritis (GCA) is the most common form of systemic vasculitis in adults, affecting preferentially medium-large size arteries. Here we report a case of a female with a diagnosis of GCA based on temporal artery biopsy, successfully treated with tocilizumab, a humanized anti-interleukin-6 receptor antibody.


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.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 15.2-16
Author(s):  
O. Argyropoulou ◽  
D. Palamidas ◽  
N. Georgantzoglou ◽  
E. Karatza ◽  
E. Xingi ◽  
...  

Background:Giant cell arteritis (GCA) represents the most prevalent form of systemic vasculitis in elderly, characterized by a remarkable heterogeneity in terms of clinical and histological phenotype, the pathogenetic mechanisms and treatment selection (1). An interplay between cells of both innate and adaptive immunity, appear to govern the pathophysiologic mechanisms (2). Among the different cellular populations, neutrophils may hold a central role in GCA pathogenesis, since they are present in abundance in tissue injury (3,4). Most importantly, they are a source of neutrophil extracellular traps (NETs) that may deliver immunocompetent substances, necessary for the perpetuation of the inflammatory response (5). The detection and function of NETs have not been studied in GCA.Objectives:To explore the presence and clinical significance of NETs in temporal artery biopsies (TABs) of patients with GCA.Methods:Ten patients with GCA [5 with limited cranial vasculitis (CV) and 5 with associated generalized large vessel vasculitis (LVV), as defined by 18F-fluorodeoxyglucose (FDG) positron-emission tomography with computed tomography (PET/CT)] and 8 patients with polymyalgia rheumatica (PMR) were studied. GCA and PMR patients fulfilled the 1990 ACR and 2012 EULAR/ACR provisional classification criteria, respectively. The presence, location, quantitation and decoration of NETs with IL-6, IL-1β, and IL-17A were assessed in TABs at the time of disease diagnosis by tissue immunofluorescence and confocal microscopy. Quantification of NETs in tissue sections was performed using the Imaris v.9.3 software that counts the total measure volume instead of only the projection area (6). Serum levels of IL-6 and IL-17A around the time of tissue biopsy were also evaluated in all patients.Results:All temporal artery biopsies from GCA patients had NETs located mainly in the adventitia, adjacent to the vasa vasorum, whereas TABs from PMR patients had no NET structures. LVV was associated with a higher NETs to total tissue volume ratio, compared to CV-GCA [p=0.0317]. NETs decorated with IL-6 were present in TABs of all LVV and 3 of 5 CV-GCA patients, while IL-17A positive NETs were observed in all GCA patients. IL-1β-positive NETs were not detected in any GCA patient. No relation was found between serum IL-6 and IL17A levels and NETs containing IL-6 and/or IL-17A.Conclusion:NETs bearing IL-6 and IL-17A cytokines are present in inflamed GCA-TABs. IL-6 positive NETs are associated with the LVV phenotype and might be useful as a tissue biomarker for disease severity and extent.References:[1]K. S. M. van der Geest et al. Arthritis Rheumatol, 2018;70:1366-76.[2]C. Salvarani et al. Lancet, 2008;372:234-45.[3]D. Chatelain et al. Ann Rheum Dis, 2009;68:84-8.[4]S. Nadkarni et al. Circ Res, 2014;114:242-8.[5]V. Mutua et al. Clin Rev Allergy Immunol, 2020.[6]S. V. Costes et al. Biophys J, 2004;86:3993-4003.Disclosure of Interests:None declared


2019 ◽  
Vol 57 (4) ◽  
pp. 341-344
Author(s):  
Andra Chiriac ◽  
Camelia Badea ◽  
Cristian Băicuș

Abstract Giant cell arteritis is a common systemic vasculitis affecting the elderly, with maximum prevalence in the 7th decade of age, targeting aortic derived medium and large vessels of the neck and head. Diagnosis is established on a biopsy specimen of the temporal artery wall, through pathological confirmation of panarteritis, typically characterized by mononuclear cell infiltrate, with the 1990 ACR criteria often used in clinical practice. We present the case of a patient with a new onset headache and systemic inflammation, who did not fulfil the classical diagnostic criteria, nor did the temporal artery biopsy (TAB) provide a positive result. However, the ultrasonographical features, clinical evolution and response to corticosteroid therapy confirmed the diagnosis. This patient had bilateral presence of the halo sign on color duplex ultrasonography (CDUS), cited as a highly specific feature, when compared to the ACR criteria as a standard reference. We employed its positive likelihood-ratio (LR+) of 43 as previously estimated, while considering a low pre-test probability for a positive diagnosis (15%), to calculate a post-test probability of 88%, leading to our decision to treat him as having giant cell arteritis. Remission of the headache and rebound phenomena when tapered off steroid therapy substantially contributed to the positive diagnosis, underlining the importance of future studies needing to use clinical evolution as a reference standard.


EMJ Radiology ◽  
2021 ◽  
Author(s):  
Patricia Harkins ◽  
Richard Conway

Giant cell arteritis (GCA) is the most common systemic vasculitis. In the past two decades there have been significant advancements in our understanding of the pathophysiological mechanisms underlying the disease, and consequently the management of GCA is evolving. GCA is a medical emergency because when left untreated it can lead to devastating complications including irreversible visual loss. Thus, prompt diagnosis is imperative to ensure appropriate treatment and prevent ischaemic events. However, uncertainty remains over diagnostic pathways, including appropriate modalities and standardisation of findings. Temporal artery biopsy has been considered the gold standard diagnostic test but has significant limitations in terms of false negative results. In recent times, several new diagnostic modalities have been proposed in GCA including temporal artery ultrasound, CT angiography, magnetic resonance angiography, and PET. In this paper, the authors review the advantages and limitations of current diagnostic modalities in GCA.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 95.3-95
Author(s):  
A. Sachdev ◽  
S. Dubey ◽  
C. Tiivas ◽  
M. George ◽  
P. Mehta

Background:A number of centres are now running fast track pathways for diagnosis and management of Giant cell arteritis with ultrasound as the first port of call for diagnosis1. Temporal artery biopsies (TABs) have become the second line of investigation, and it is unclear how useful TAB is in this setting.Objectives:This study looked at accuracy of Temporal artery biopsy (TAB) in patients with suspected Giant Cell arteritis (GCA) with negative/inconclusive ultrasound (U/S) and how duration of treatment on steroids prior to these investigations and arterial specimen size affected it.Methods:Prospective study of all patients with suspected GCA referred for TAB when U/S was negative or inconclusive, as part of the local fast-track pathway (Coventry). Database included clinical findings, serological work up, U/S and TAB results and treatment. Sensitivity and specificity of U/S and TAB was calculated and compared based on duration of treatment with steroids.Results:One hundred and nine patients were referred for TAB via Coventry fast-track-pathway. The sensitivity of U/S in this cohort of patients was 9.08% and specificity was 93.33%. After 3 days of steroid this was 0% and 100% respectively. For TAB when done within 10 days of starting steroids, this was 65% and 87.5% respectively. After 20 days of steroids this was 0 % and 100%. The sensitivity and specificity was 20% and 85% when arterial specimen size was 11-15mm and 47% and 100% when specimen size was 16 mm or more. Sensitivity and specificity of U/S of 644 suspected GCA patients was 48% and 98%.Conclusion:Our study demonstrates that TAB plays a relevant role in GCA fast-track-pathways, when U/S is negative/inconclusive. TAB was more sensitive than U/S in this cohort of patients, but overall sensitivity of U/S was higher when calculated for all patients suspected with GCA. Both remain useful tests if performed early. TAB specimen size should ideally be 16mm or more and done within 10 days of starting steroids.References:[1]Jonathan Pinnell, Carl Tiivas, Kaushik Chaudhuri, Purnima Mehta, Shirish Dubey, O38 The diagnostic performance of ultrasound Doppler in a fast-track pathway for giant cell arteritis,Rheumatology, Volume 58, Issue Supplement_3, April 2019, kez105.036,https://doi.org/10.1093/rheumatology/kez105.036Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 682.1-682
Author(s):  
S. Chrysidis ◽  
U. Møller Døhn ◽  
L. Terslev ◽  
U. Fredberg ◽  
T. Lorenzen ◽  
...  

Background:Giant Cell Arteritis (GCA) is one of the most common systemic vasculitis. Temporal artery biopsy (TAB) has been the standard test to confirm the diagnosis of GCA. However, TAB has a lower sensitivity than clinical diagnosis and up to 44% of biopsy-negative patients are clinically diagnosed as having GCA.In a recent meta-analysis of the diagnostic performance of ultrasound (US) in GCA the sensitivity was 77 % (1). The included studies were performed by expert groups in single centres. In the to date only multicentre study (TABUL) investigating the diagnostic accuracy of US compared to clinical diagnosis after 6 months the sensitivity was lower (54%) (2)Objectives:To evaluate the diagnostic accuracy of vascular US compared to TAB in a multicentre study.Methods:In three Danish centres patients suspected for GCA were included during a period of two years. At baseline, clinical and laboratory data were collected and vascular US of temporal, facial, common carotid and axillary artery were performed. The US examinations were performed with high frequency transducers (15-18 MHZ) and followed by a TAB. All ultrasongraphers had participated in the same standardized US educational program and were blinded to clinical and laboratory data. An external expert blinded to clinical and laboratory data evaluated all images and made the final US diagnosis.A positive sign for vasculitis in cranial arteries was defined as a hypoechoic intima media complex (IMC) thickening (halo sign) and a positive compression sign. A homogeneous IMC increased thickness in axillary artery of ≥1mm and in common carotid artery ≥1.5mm was defined as vasculitis.The consultant rheumatologist’s diagnosis at 6 months after initial presentation was considered as the reference standard for the diagnosis of GCA.Results:During the recruitment period, 112 patients were included, 59% females, mean (SD) age 72.4(7.9) years, among which 91(81.3%) fulfilled the ACR 1990 classification criteria for GCA. 92% of the patients reported a newly emerged localized headache, while 49 (43.8%) experienced polymyalgia rheumatic symptoms.TAB was positive in 46(41.1%) and inconclusive in 6 patients, who were excluded from the analysis. Mean (SD) duration of glucocorticoid therapy prior to US and TAB was 0.91(1.55) and 4.02(2.61) days, respectively. In 62 patients, the final diagnosis was GCA.In all patients with a positive TAB, the US of the temporal artery was also positive for GCA. Of 19 cases with positive US and negative TAB, 12 were clinically diagnosed with GCA of whom 6 had isolated large vessel involvement on US. Among 41 patients with both negative US and TAB, 4 were clinically diagnosed with GCA (Box 1)US had a sensitivity of 93% and specificity of 84% for the diagnosis of GCA, while the sensitivity for TAB was lower (74%) with a specificity of 100%. For the diagnosis of GCA, US had a PPV of 89.2 % and a NPV of 90.2%, while for TAB the PPV was 100% and the NPV 73.3%.Conclusion:US evaluation of the temporal, facial and selected supraaortic arteries performed by trained ultrasonographers can replace biopsy in the diagnosis of GCA.Box.1References:[1]Duftner C, Dejaco C, et al. Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: a systematic literature review and metaanalysis informing the EULAR recommendations. RMD Open 2018;4:e000612.[2]Luqmani R et al. The Role of Ultrasound Compared to Biopsy of Temporal Arteries in the Diagnosis and Treatment of Giant Cell Arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study. Health Technol Assess 2016;20:1_238.Disclosure of Interests:stavros chrysidis: None declared, Uffe Møller Døhn: None declared, Lene Terslev Speakers bureau: LT declares speakers fees from Roche, MSD, BMS, Pfizer, AbbVie, Novartis, and Janssen., Ulrich Fredberg: None declared, Tove Lorenzen: None declared, Robin Christensen: None declared, Per Søndergaard: None declared, Jakob Matthisson: None declared, Knud Larsen: None declared, Andreas Diamandopoulos: None declared


2011 ◽  
Vol 121 (S5) ◽  
pp. S264-S264
Author(s):  
Stephen V. Tornabene ◽  
Raymond Hilsinger ◽  
Raul M. Cruz

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