scholarly journals Diagnosis of giant cell arteritis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii5-iii16 ◽  
Author(s):  
Cristina Ponte ◽  
Joana Martins-Martinho ◽  
Raashid Ahmed Luqmani
Keyword(s):  
Systemic Vasculitis ◽  
Correct Diagnosis ◽  
Temporal Artery ◽  
Current Evidence ◽  
Temporal Artery Biopsy ◽  
Diagnostic Modality ◽  
Significant Toxicity ◽  
Primary Systemic Vasculitis ◽  
High Doses ◽  
Low Sensitivity

Abstract GCA is the most common form of primary systemic vasculitis affecting older people. It is considered a clinical emergency because it can lead to irreversible blindness in around 20% of untreated cases. High doses of glucocorticoids should be initiated promptly to prevent disease-related complications; however, glucocorticoids therapy usually results in significant toxicity. Therefore, correct diagnosis is crucial. For many years, temporal artery biopsy has been considered the diagnostic ‘gold standard’ for GCA, but it has many limitations (including low sensitivity). US has proven to be effective for diagnosing GCA and can reliably replace temporal artery biopsy in particular clinical settings. In cases of suspected GCA with large-vessel involvement, other imaging modalities can be used for diagnosis (e.g. CT and PET). Here we review the current evidence for each diagnostic modality and propose an algorithm to diagnose cranial-GCA in a setting with rapid access to high quality US.

scholarly journals Temporal Arteritis and Vision Loss in Microscopic Polyangiitis: A Case Report and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Alexander G. Goglia ◽  
Michael Makar ◽  
Craig Vanuitert ◽  
Vadim Finkelstein
Keyword(s):  
Vision Loss ◽  
Temporal Arteritis ◽  
Microscopic Polyangiitis ◽  
Systemic Vasculitis ◽  
Temporal Artery ◽  
Multiple Organ ◽  
The Body ◽  
Temporal Artery Biopsy ◽  
Rare Finding ◽  
Organ Systems

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. While the lungs and kidneys are the major organs affected by MPA, it is known to involve multiple organ systems throughout the body. Temporal artery involvement is a very rare finding in MPA. This report presents a patient whose initial presentation was consistent with giant cell arteritis but was ultimately found to have microscopic polyangiitis. It highlights the importance of considering alternative types of vasculitis in the differential diagnosis for patients with atypical temporal artery biopsy findings.

scholarly journals Polymialgia Rheumatica, Giant Cell Arteritis or Both?

2018 ◽  
Vol 15 (6) ◽  
pp. 51-58
Author(s):  
Adina Cociorvei ◽  
Mădălina Ababei
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Arteritis ◽  
Morning Stiffness ◽  
Systemic Vasculitis ◽  
Diagnostic Algorithm ◽  
Signs And Symptoms ◽  
Temporal Artery ◽  
Clinical Suspicion ◽  
Temporal Artery Biopsy

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.

scholarly journals Successful Treatment of a Patient with Giant Cell Vasculitis (Horton Arteritis) with Tocilizumab a Humanized Anti-Interleukin-6 Receptor Antibody

2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Alfredomaria Lurati ◽  
Luca Bertani ◽  
Katia Angela Re ◽  
Mariagrazia Marrazza ◽  
Daniela Bompane ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Successful Treatment ◽  
Interleukin 6 ◽  
Systemic Vasculitis ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Receptor Antibody ◽  
Large Size ◽  
Interleukin 6 Receptor

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.

scholarly journals 39. Atypical presentation of giant cell arteritis confirmed on brain biopsy

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Nadia Ahmad ◽  
Elizabeth Price ◽  
Areli Cuevas-Ocampo ◽  
Khin Yein ◽  
Azeem Ahmed
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Conflicts Of Interest ◽  
Systemic Vasculitis ◽  
Granulomatous Inflammation ◽  
Brain Biopsy ◽  
Temporal Artery ◽  
Cerebral Vasculitis ◽  
Temporal Artery Biopsy ◽  
History Of

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.

scholarly journals Will imaging change the diagnosis and management of giant cell arteritis?

2019 ◽  
Vol 57 (4) ◽  
pp. 341-344
Author(s):  
Andra Chiriac ◽  
Camelia Badea ◽  
Cristian Băicuș
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Systemic Vasculitis ◽  
Positive Likelihood Ratio ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Positive Diagnosis ◽  
Clinical Evolution ◽  
Acr Criteria ◽  
Test Probability

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.

scholarly journals Giant Cell Arteritis: Navigating Beyond the Headache

EMJ Radiology ◽  
2021 ◽  
Author(s):  
Patricia Harkins ◽  
Richard Conway
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Systemic Vasculitis ◽  
False Negative ◽  
Temporal Artery ◽  
Medical Emergency ◽  
Temporal Artery Biopsy ◽  
Negative Results ◽  
False Negative Results ◽  
Diagnostic Modalities

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.

scholarly journals THU0553 A HEALTH ECONOMIC ANALYSIS OF THE USE OF COLOUR DOPPLER ULTRASONOGRAPHY AS THE PRIMARY DIAGNOSTIC MODALITY IN PATIENTS WITH SUSPECTED GIANT CELL ARTERITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 517.2-517
Author(s):  
C. Mukhtyar ◽  
L. Steel ◽  
C. Jones ◽  
M. Bachmann
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Definite Diagnosis ◽  
First Line ◽  
Diagnostic Modality ◽  
Positive Diagnosis ◽  
The Cost ◽  
Test Probability

Background:EULAR has recommended ultrasonography (US) as first imaging modality for diagnosis of Giant Cell Arteritis (GCA)1. For patients with a high pre-test probability who have a negative scan, the recommendation is to use another diagnostic modality like temporal artery biopsy (TAB) to make a diagnosis1. We know that a fast-track pathway incorporating US, results in better clinical outcomes2; but there are little data on the health-economics of this approach. Since 2017, we have used ultrasonography as the primary diagnostic modality for suspected GCA. In patients with a high pre-test probability with a negative ultrasonography, we perform a temporal artery biopsy.Objectives:To compare the cost of investigating GCA using first-line US and second-line TAB the use of TAB only. To compare the cost per definite diagnosis of GCA.Methods:Number of cases from 2007-2009 and 2017-2019 were calculated by the number of TAB performed and number of referrals to hospital GCA clinic, respectively. Costs of the procedure were calculated as per the nationally agreed tariff by the United Kingdom National Health Service. For ease of comparison, we used the 2018/19 tariff (£1284/TAB; £51 for US)Results:In 2007-2009, 162 cases were referred to clinic and had a TAB, of which 86 were positive. No cases had US. The 2018/19 corrected cost was £208008; the cost per positive diagnosis was £2418.70 (Table 1).In 2017-2019, 419 patients were referred to the GCA clinic, 416 of whom had US for diagnosis. 3 individuals had a TAB as the first diagnostic modality and 66 others were referred for a TAB because of a high pre-test probability and negative US. The 2018/19 corrected cost of this pathway was £109812 and the cost per positive diagnosis was £773.32 (Table 1).Table 1.Numbers of cases investigated for suspected GCA by TAB or US and the costs corrected to 2018/19 UK NHS tariff.YearsNo of referralsNo of TABNo of USNo of patients with GCATotal costCost of making 1 positive diagnosis2007-09162162086£208008£2418.702017-1941969416142£109812£773.32If all cases in 2017-2019 had a TAB for suspected GCA, the 2018/19 corrected cost would have been £537996. The estimated 2018/19 corrected savings in our center was £142728/year. The estimated 2018/19 corrected savings per definite diagnosis of GCA has dropped by £1645.37 (Table 1).Conclusion:The EULAR recommendation of using first-line US for diagnosis of GCA followed by a TAB in cases with uncertain diagnosis after US, is highly cost-effective in the UK, resulting in cost savings of >£140K per year.References:[1]Dejaco C, et al. Ann Rheum Dis. 2018 May;77(5):636-643.[2]Diamantopoulos AP, et al. Rheumatology (Oxford). 2016 Jan;55(1):66-70.Disclosure of Interests:None declared

scholarly journals AB0377 CLINICAL REVIEW OF 19 CASES WITH IDIOPATHIC AORTITIS – A SINGLE CENTRE STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1215.2-1216
Author(s):  
M. J. Villar ◽  
S. Sangle ◽  
D. D’cruz
Keyword(s):  
Abdominal Aorta ◽  
Systemic Vasculitis ◽  
Temporal Artery ◽  
Patient Characteristics ◽  
Response To Treatment ◽  
Temporal Artery Biopsy ◽  
Surgery Patient ◽  
Single Centre Study ◽  
Aneurysm Dissection

Background:Aortitis is a group of disorders leading to inflammation in the aorta. When aortitis has no clinical evidence of systemic vasculitis, it is called idiopathic aortitis or clinically isolated aortitis (CIA).There is no consensus on the management of CIA.Objectives:Our purpose is to describe our cohort of patients with CIA and their response to treatment.Methods:This is a retrospective analysis of 19 patients with CIA. All records of patients with CIA were analyzed and demographic variables, comorbidities, symptoms, images, histology, treatmentreceived, outcome and mortality were recorded. The description of quantitative variables was made using the median and the interquartile range (IQR).Results:Nineteen patients were analyzed. Diagnosis was made by imaging in 18 (94.7%), one patient was diagnosed by histology after aortic root surgery. Patient characteristics are detailed in Table 1.The median duration of follow- up was 38 months (IQR 43). Seven patients (36.8%) had constitutional symptoms including fever, weight loss, sweats and fatigue. 4 (21.05%) presented with back or abdominal pain for a mean duration of 3 months (SD 0.81) before the diagnosis. In 8 (42.1%) patients the diagnosis was made incidentally.All patients had negative treponema, hepatitis B, hepatitis C and Human Immunodeficiency Virus (HIV) serology.All patients had negative autoimmune serology included ANCA. Interestingly 4 (21.05%) had positive lupus anticoagulant without other manifestation of antiphospholipid syndrome.The type of aortic involvement was aortitis in 10 patients (52.6%), inflammatory aneurysm in 8 (42.1%) and dissection in 1 (5.2%).Seven (36.8%) patients had thoracic and abdominal aorta involvement, 6 (31.5%) only thoracic aorta and 6 (31.5%) only abdominal aorta.Aortic histology was obtained in 5 patients, 2 had necrotizing arteritis with giant cell pattern and 2 had lymphoplasmacytic pattern. Temporal artery biopsy was done in one patient and the result was negative for GCA.All patients received corticosteroids as a remission inducing agent, 12 (63.1%) received methotrexate and 2 (10.5%) mycophenolate. 2 patients died (10.5%).The median prednisone dose at the beginning was 20 mg (IQR 20) and at remission was 5 mg (IQR 20). 41.6% (5/12) of patients treated with steroids plus methotrexate were able to stop steroids without reactivation over a median follow-up time of 23.5 months (IQR 31). (Table 2.)Details of treatment, ESR and CRP pre and post treatment is shown in Figure 1.Seven patients (36.8%) had follow-up imaging, none of them showed active inflammation, new aneurysm, dissection or disease progression.Conclusion:Treatment of isolated aortitis with steroids and methotrexate was effective resulting in clinical and laboratory improvement and allowed cessation or decrease in steroids to 5 mg or less in 83% of patients. This therapeutic approach may be useful in patients with CIA.References:[1]Cinar, I., Wang, H., & Stone, J. R. (2017). Clinically isolated aortitis: pitfalls, progress, and possibilities. Cardiovascular Pathology, 29, 23–32.Disclosure of Interests:None declared

scholarly journals P040 An unusual case of giant cell arteritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson
Keyword(s):  
Inflammatory Markers ◽  
Illicit Drugs ◽  
Unusual Case ◽  
Temporal Arteritis ◽  
White Cell Count ◽  
Healthcare Providers ◽  
Temporal Artery ◽  
Low Grade ◽  
Temporal Artery Biopsy ◽  
The Right

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.

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