Temporal Artery Biopsy Research Articles

Mönckeberg sclerosis with giant cells as a masquerade of giant cell arteritis.

Giant cell arteritis (GCA) is the most common type of vasculitis in adults, which is classified as a large/medium vessel vasculitis. It has a predilection for the ophthalmic circulation and extracranial carotid system. Temporal artery biopsy specimens can show the presence of inflammatory multinucleated giant cells. Here, we report just the third case of Mönckeberg sclerosis with multinucleated giant cells affecting the temporal artery and mimicking GCA. This rare finding in the evaluation of a common vasculitis is important for rheumatologists to be aware of and emphasizes close collaboration between clinicians and pathologists.

A Predictive Model for Temporal Artery Biopsy in the Setting of Suspected Giant Cell Arteritis: A Validation Study.

A previously published predictive model based on threshold parameters for erythrocyte sedimentation rate, c-reactive protein, and platelet count demonstrated that 40% of patients who underwent biopsy may not have required it. The current study was performed to evaluate the model's performance on an independent data set. This is a retrospective consecutive series of patients undergoing temporal artery biopsy (TAB) in a single health region in Canada. The model was applied to a multicenter cohort of patients undergoing TAB by a variety of surgical services. A centralized pathological database serving multiple institutions and surgical services was used to identify patients undergoing TAB. Over a 7-year period, patients undergoing TAB were identified via a central pathological database. Those who had concurrent illnesses which would likely affect erythrocyte sedimentation rate, c-reactive protein, and platelet count, patients on steroids for >2 weeks by the time of biopsy, and those with missing serum markers were excluded. The previously developed model was applied to the 222 patients enrolled. The model correctly identified 29% of patients with a pretest probability of 0% for a positive biopsy and 9% with a pretest probability of 100%, suggesting that in total, 38% of patients could have avoided TAB. The results of this independent data set support the previously published predictive formula. Utilizing a simple, clinically applicable predictive model of the pretest probabilities, approximately 38% of TAB currently being performed may be avoided. The results suggest that evaluation with a prospective multicentre study would be appropriate.

Vasculitis de gran vaso: arteritis de células gigantes y arteritis de Takayasu

Large-vessel vasculitis predominantly affects the aorta and its branches and is classified into two main entities: giant cell arteritis (GCA) and Takayasu arteritis (TKA). GCA usually presents with new-onset temporal headache, jaw claudication and scalp hypersensitivity. Some patients also have visual symptoms (amaurosis fugax and anterior optic neuritis) and systemic manifestations such as polymyalgia rheumatica, fever and weight loss. Temporal artery biopsy is an important diagnostic aid. Other tools include temporal artery ultrasound, positron emission tomography combined with computed tomography (PET-CT) and magnetic resonance angiography (MRA). Glucocorticoids (GC) are the mainstay of treatment for GCA, reserving combining with tocilizumab or methotrexate for patients with recurrences or significant corticosteroid-related toxicity. The onset of symptoms in TKA tends to be subacute, often resulting in diagnostic delay. A clinical diagnosis of TKA can be based on suggestive clinical findings (constitutional symptoms, carotid artery disease, hypertension, decreased or absent pulses and/or arterial murmurs in patients under 40 years of age) and an imaging test showing vascular narrowing of the aorta and/or its primary branches. Angio-CT helps to detect complications of vasculitis and PET/CT to assess response to treatment. Systemic GCs are the mainstay of treatment for TKA, and the early combination of a steroid-sparing drug due to the high rate of relapse.

Giant cell arteritis

Giant cell arteritis (GCA) is the most common vasculitis of older age. It usually affects the branches of carotid arteries, especially temporal and ophthalmic artery. In addition to genetic predisposition, environmental factors also contribute to the development of the disease. Interleukin 6 (IL-6), whose level correlates with inflammatory activity, is one of the key cytokines in the pathogenesis of the disease. Sterile inflammation of the artery wall with intimal hyperplasia and the development of occlusion leads to ischemia which is responsible for the onset of symptoms. The clinical picture is dominated by headache together with general symptoms. Sudden loss of vision may occur due to anterior ischemic optic neuropathy (AION). Frequently, signs of rheumatic polymyalgia are also present. Markedly high erythrocyte sedimentation rate (ESR) and elevated C reactive protein (CRP) are typical. The diagnosis is based on clinical and laboratory findings, temporal artery biopsy, and imaging examinations. Prompt diagnosis is crucial for timely and adequate treatment as well as prevention of early and late complications (blindness, arterial aneurysm or dissection). Until recently, treatment has been limited to the use of glucocorticoids and conventional immunomodulating drugs. This review paper outlines new therapeutic approaches arising from a better knowledge of pathophysiology. Insights from recent studies led to corrections of GCA treatment guidelines (as did the Croatian Society for Rheumatology in 2018) by recommending IL-6 inhibitor tocilizumab for patients with refractory or relapsing disease, and initially in patients at increased risk for complications. Clinical trials are underway that could deliver other therapeutic options in the near future.

Giant Cell Arteritis

Giant cell arteritis (GCA), also referred to as temporal arteritis, is a variant of large-vessel vasculitis. GCA should be considered in the differential diagnosis in patients aged >50 years, who present with headache, abrupt onset of visual disturbances, unexplained fever, a high erythrocyte sedimentation rate, and high serum C-reactive protein levels. Diagnosis of GCA is based on accurate interpretation of laboratory data, temporal artery biopsy findings, and imaging study results. Imaging modalities used for GCA include positron emission tomography, computed tomography (CT), CT angiography, and conventional magnetic resonance angiography. Biopsy is the gold standard for diagnosis of GCA. Glucocorticoids (GCs) are used as standard treatment to induce remission and also for maintenance therapy. However, clinicians should be aware of the adverse effects of GC treatment, including hyperglycemia, an immunocompromised state, and delirium. If adverse effects outweigh the benefits of GC treatment, it is necessary to consider switching to or adding an immunosuppressant to the therapeutic regimen.

A Promising Alternative to Temporal Artery Biopsy for GCA

A Promising Alternative to Temporal Artery Biopsy for GCA

P37: THE IMPACT OF TEMPORAL ARTERY BIOPSY AT A UK TERTIARY PLASTIC SURGERY UNIT

Abstract Introduction Guidelines recommend temporal artery biopsy (TAB) for patients suspected of having giant cell arteritis (GCA). We evaluated the impact of TAB on the diagnosis and management of patients with suspected GCA at a tertiary plastic surgery unit. Method A retrospective review of all TAB procedures performed at our centre over 7 years was performed. One hundred and one patients were included in the study. Patients were classified into 3 diagnostic groups: confirmed (positive TAB), presumed (negative TAB with high clinical suspicion) and unlikely (negative TAB with low clinical suspicion). The clinical presentation and management for each group were compared. Result The average American College of Rheumatology (ACR) score was 3.07. The number of patients with an ACR score of ≥3 before TAB was 72 (71.3%) and remained the same after TAB. The number of patients who remained on steroid therapy was lower in the group with an unlikely diagnosis of GCA compared to the group with a confirmed diagnosis (p<0.05). Conversely, there was no significant difference in steroid therapy between those with a presumed and confirmed diagnosis (p>0.05). Conclusion This study found a significant difference in steroid treatment between those with confirmed GCA and those where the diagnosis was unlikely showing that TAB may support decisions regarding steroid therapy. However, TAB was inappropriately requested for patients whose pre-TAB ACR score was ≥3 as this score is sufficient for the diagnosis of GCA. Therefore, the use of TAB should be limited to cases of diagnostic uncertainty. Take-home message while temporal artery biopsy has a role to play in the diagnosis of GCA, its use should be limited to cases of diagnostic uncertainty and not requested for every patient with a suspicion of Giant Cell Arteritis.

P006 The potential of colour Doppler ultrasound in reducing temporal artery biopsies in suspected giant cell arteritis

Abstract Background/Aims Giant cell arteritis (GCA) is an emergency. The initial treatment with high dose glucocorticoids (GC) is often started on clinical suspicion without waiting for temporal artery biopsy (TAB) results. Colour Doppler ultrasound (CDUS) is a simple, non-invasive test which is readily available. However, like any other ultrasound, it is operator dependent. Non-compressible ‘halo sign’ is the most specific abnormality on CDUS. British society for Rheumatology (BSR) guidelines advises to avoid TAB in patients with low clinical probability and negative CDUS as well as in high clinical probability and positive CDUS. Methods We adopted the quality improvement methodology for assessment. Retrospective data of suspected GCA patients was collected over the last two years. CDUS was introduced to investigative plan midway, after eleven months. Two rheumatology consultants were trained in CDUS. Results were compared before and after the introduction of ultrasound as a diagnostic tool. In collecting the data, our main focus for documentation was based on clinical symptoms, CDUS and TAB results. Patient were divided into high, medium and low probability groups based on clinical assessment by a rheumatologist. Final diagnosis was decided on the basis of clinical assessment at 6 months. Results It was a retrospective review from January 2018 to November 2019, 101 patients were referred with suspected GCA. Median age was 72 years (50 - 91 years) with male to female ratio of 1:3. Thirty five patients were referred in the first 11 months and 28.6% were diagnosed with GCA. CDUS and TAB was done in 20% and 49% of patients respectively. Sixty six patients were referred in the next 12 months after CDUS was introduced and 21.2% were diagnosed as GCA. CDUS and TAB were done in 82% and 38% of the patients respectively. We reviewed all TABs in the second phase of QIP (38%). As per current BSR guideline, 8 TABs could have been avoided in patients with positive CDUS and high probability of GCA or negative CDUS and low probability of GCA. Even if we deduct these 8 TABs from total of 25, 26% of our suspected GCA referrals would still require TAB for diagnostic workup. Conclusion After the routine introduction of CDUS, the percentage of patients requiring TAB has declined. Approximately one fourth patients would still require TAB as per BSR guidelines. To improve the clinical relevance of biopsies further we recommended; the routine use of GCA probability score, improve CDUS skills and arrange availability of urgent slots in clinic for CDUS. We also noticed that the number of patients referred has almost doubled. This might be due to better education and awareness at the primary and secondary care level which was done as part of the project. Disclosure A.A. Sidhu: None. A. Nandagudi: None. A. Bharadwaj: None.

P013 Driving improvement through audit: impact of 2017 regional audit and survey upon giant cell arteritis services in 2020

Background/Aims In 2017 an audit and survey of giant-cell arteritis (GCA) services were conducted across northwest England (reported previously). This re-survey in 2020, following publication of revised BSR guidance, sought to identify what changes were made in the intervening period, and provided the opportunity to assess the impact of COVID-19. Methods Rheumatologists from 16 hospitals in northwest England were invited to complete a survey in July 2020. Questions focused on service provision for GCA, including pathways, diagnostics and steroid prescription. Results Responses were received from 14/16 sites in 2017, and 15/16 in 2020. 9/15 (60%) sites reported that the 2017 audit and survey prompted changes to GCA services, with two (13%) stating that it clarified the need for implementation of existing plans. Two sites had a GCA pathway in 2017. Four of the seven sites who committed to introducing one have now done so, bringing the total in 2020 to six. Eight of the nine remaining sites plan to implement one, six with a specific date within six months. Six (40%) have completed additional local audit/QI since 2017. Temporal artery (TA) ultrasound (US) is now available in an additional four sites, bringing the total to 6/15 (40%) in 2020. Two sites reported improvement in both time between first rheumatology consultation and TA biopsy, and time to receive results (now <7 days for each task in 6/15 (40%)). Six additional sites reported providing leaflets on steroids routinely, bringing the total in 2020 to 12/15 (80%), versus 6/14 (43%) previously. Four sites (27%) now have a database of GCA patients (one in 2017). There was no major change in sites having a standard protocol for steroid taper (n = 8 2017; n = 7 2020, 89% and 100% of whom respectively use BSR guidance), nor in the number of patients routinely provided steroid cards (six in 2017; five in 2020). The three sites who do not report giving leaflets on steroids routinely, all had a pathway. 8/15 (53%) reported COVID-19 having an adverse effect upon services, including: reduced access to diagnostics (n = 7: TA US, biopsy, and PET-CT); delayed appointments (n = 4); delayed referrals (n = 3). The tertiary referral centre reported an improvement because access to tocilizumab was facilitated by a relaxation of rules by NHS England. Conclusion The original audit and survey of current GCA practice in 2017 highlighted areas for improvement for each site, and regionally. Sites contributing to this re-survey report that the exercise stimulated them to improve their current care. The 2017 exercise showed a strong correlation between reported practice (survey) and actual practice (audit), leading us to have confidence that responses provided a true picture of care. This work demonstrates the power of audit to drive improvement, at a regional level. Disclosure C.A. Sharp: None. J. Little: None. A. Shahbaz: None. E.F. Wood: None. S. Wig: None. P. Watson: None. S. Varughese: None. L. Teh: None. P. Shah: None. C. Saleh: None. L. Ottewell: None. L. Newton: None. S.R. Moore: None. E. McCarthy: Consultancies; E.M. has received consultancy fees from Chugai. E. MacPhie: None. K. Lazarewicz: None. J. Brockbank: None. J. Bluett: None. L. Mercer: None.

O16 Positron emission tomography - computed tomography: an important diagnostic tool in the giant cell arteritis fast track pathway

Abstract Background/Aims Giant cell arteritis (GCA) is a systemic vasculitis affecting the temporal arteries and large vessels, including the aorta, in 25% of cases. Incidence of vertebral artery involvement is unknown likely owing to the reliance on conventional diagnostics including temporal artery biopsy (TAB) and temporal/axillary ultrasound which evade the vertebral arteries. These tests lose sensitivity soon after steroid initiation. Early specialist assessment and diagnostics, as part of a fast track pathway (FTP), can overcome this challenge. We present the first 12 months data from our FTP. To our knowledge this is the first GCA-FTP in the UK to utilise positron emission tomography - computed tomography (PET-CT) as an early diagnostic tool so we present a unique cohort of patients. Methods Cohort 1: 40 patients, diagnosed between 2015-2019, before FTP implementation. Time from steroid initiation to temporal artery ultrasound (TAUS) and TAB was recorded. Cohort 2: 35 patients referred after pathway implementation. 21 had a positive diagnosis. Time from steroid initiation to diagnostic test was recorded. Results Cohort 1: 26 (65%) patients had TAB; 3 (7.5%) had TAUS. The average time from starting steroid to investigation was 6 and 2 days respectively. Cohort 2: 17 (48%) had TAB, 30 (86%) had TAUS, 28 (80%) had PET-CT. In confirmed diagnoses, time from steroid initiation to investigations was 6.6 days, 1 day and 2.5 days respectively. In negative diagnoses time frames were 11.5, 1.5 and 2.3 days respectively. Table 1 shows sensitivity and specificity of these tests. Table 1. Sensitivity and specificity comparison between diagnostic tests Conclusion Combining TAUS and PET-CT allows for high diagnostic accuracy without the need for invasive biopsy. 24% of patients had negative or inconclusive ultrasound and/ or negative biopsy but confirmed vasculitis on PET-CT. 50% of this cohort had vertebral involvement only. PET-CT has an important role in detecting extracranial particularly vertebral arteritis, where biopsy and ultrasound are unreliable. Our pathway design with the inclusion of PET-CT, enables us to capture all patients with GCA and satisfy NHS England criteria for tocilizumab use in refractory GCA. Continued evaluation of PET-CT and its role in predicting vascular complications is required. Disclosure D.R. Ludwig: None. V. Morris: None. A. Shashidhara: None. S. Voo: None. R. Reshat: None.

P206 Rethinking old biomarkers: novel predictors of giant cell arteritis

Abstract Background/Aims Giant cell arteritis (GCA) is a large vessel vasculitis mainly affecting the arteries of the head and neck which, if untreated, may lead to permanent vision loss. Glucocorticoids are highly effective at turning off inflammation but come with toxic side effects, making prompt diagnosis essential. There is no gold-standard investigation for GCA. Although specific, temporal artery biopsy (TAB) is only positive in approximately 25% of cases making it problematic as a rule-in diagnostic test. Vascular ultrasound may aid diagnosis but there is a rapid reduction in sensitivity with glucocorticoid use and it is not yet universally available. Diagnosis therefore requires the integration of clinical judgment with blood tests measuring inflammation, imaging, and biopsy. Aims:Identify which components of the history, examination and laboratory findings are most predictive of a positive diagnosis of GCA in the local region of Yeovil District Hospital, and to investigate the usefulness of alternative blood biomarkers. Methods Data was collected from GCA clinic attendances between August 2018 and February 2020 using electronic notes, clinic letters and the pathology system. Predictive values, sensitivity, specificity, and receiver operating characteristic (ROC) curves were calculated for each individual parameter and for groups of parameters. Results Ninety-one patients presented to GCA clinic in the 18 months studied. Median age was 71 and 73% were female. 56 patients with suspected disease went on to have TAB, of which 38/56 (68%) were of adequate length ( &amp;gt; =10mm), and of those, 12/38 (32%) confirmed a diagnosis of GCA. 43/91 (47%) patients were ultimately diagnosed biopsy proven or suspected GCA. Headache was the most common presenting feature (88%) followed by raised ESR (55%), raised CRP (53%), visual disturbance (44%), scalp tenderness (33%), jaw claudication (31%), PMR symptoms (27%) and temporal artery abnormalities (20%). Headache and raised CRP+/-ESR were the most sensitive markers (91% and 100%, respectively). They were, however, the least specific (4% and 36%). Temporal artery abnormality was the most specific finding (81%). ROC analysis revealed that the best-performing biomarkers were monocytes (area under the ROC curve (AUC) of 0.81) and platelets (AUC 0.80), which were superior to jaw claudication, the best-performing classical biomarker (AUC 0.68). Platelets above 450 x 109/L had a specificity of 96% with a likelihood ratio of 10.9. Monocytes above 0.45 x 106/L had sensitivity and specificity of 100% and 67%, respectively. Conclusion GCA cannot be accurately predicted by any single feature. In this cohort, absence of headache with a normal CRP+/-ESR ruled out GCA. Platelets and monocytes performed better than all the classical parameters associated with GCA. Validation of these biomarkers in a larger cohort is now needed to ascertain cut-off points which may help to develop a more accurate method to predict cases of GCA. Disclosure T. Somoskeoy: None. A. Bourn: None. S. Knights: None. B. Mulhearn: None.

P038 Is it all in the eyes?

Abstract Background/Aims The ocular manifestations of giant cell arteritis (GCA) and ocular myasthenia gravis (OMG) have significant overlap, which can lead to clinical uncertainty in the absence of awareness and routine diagnostic testing. Methods We present the case of a 63-year-old male patient with a strong family history of autoimmune disease. He presented to the Rheumatology department in 2009 with symptoms of polymyalgia rheumatica (PMR) and was treated successfully with a reducing regime of Prednisolone. In 2013, he presented to the Ophthalmology department with blurred vision and diplopia. Giant cell arteritis was suspected and a high dose of Prednisolone was started. His temporal artery biopsy was normal and inflammatory markers were not raised, but there was a complete resolution of his symptoms since starting steroids. Under the care of the Rheumatology department, reducing regimes of Prednisolone were attempted and a steroid sparing agent, Methotrexate was introduced. Unfortunately, he continued to have recurring symptoms of blurred vision and diplopia, particularly at Prednisolone doses less than 20mg. Over the course of a few years, there was a progression of his ocular symptoms. He developed a horizontal diplopia, monocular ptosis and a 4th nerve palsy. He was referred back to the Ophthalmology department, where alternate diagnoses were investigated. Anti-acetylcholine receptor antibodies (AChR) were strongly positive at 35 mU/L (normal &amp;lt;0.44 mU/L). MRI head was normal, and CT chest showed no thymoma. Single fibre EMG confirmed severe OMG. Our patient was subsequently commenced on Pyridostigmine, with which symptoms improved. Results The literature on OMG mimicking GCA is sparse. The prevalence of an exclusively ophthalmic presentation in patients with GCA is 1 in 5. Ocular signs and symptoms in GCA can vary drastically due to the propensity of the disease to affect different structures within the eye. Transient and permanent visual loss, affects 10-30% of GCA patients due to anterior ischemic optic neuropathy. Diplopia affects 5% of GCA patients. In comparison, OMG can be the only presenting feature in 15% of patients with Myasthenia Gravis, and is characterised by unilateral ptosis, oculomotor paresis and binocular diplopia. Conclusion This case highlights the clinical similarities of OMG and GCA, and that OMG should be considered as a differential diagnosis when the clinical picture of GCA does not truly fit. In patients presenting with GCA with ocular symptoms, normal inflammatory markers and no systemic features, OMG should be considered and AChR antibody testing be performed. More awareness of OMG is needed. We also propose that OMG should be included as a differential diagnosis, in the British Society of Rheumatology guidelines for GCA. Disclosure A. Gharatya: None. J. Wajed: None. N. Cernovschi: None. D. Christidis: None.

P204 An evaluation of a daily fast-track diagnostic ultrasound service for patients with suspected new onset giant cell arteritis during the COVID-19 pandemic

Background/Aims COVID-19 has reinforced the necessity for a faster and more accurate diagnostic pathway for suspected giant cell arteritis (GCA) patients so that unnecessary use of glucocorticoids can be avoided/minimised. During the COVID-19 pandemic, we replaced a twice weekly service with a 5 day per week ultrasound-based fast-track pathway within a secondary/tertiary hospital. The aim was to determine the impact of a more frequent diagnostic service for GCA, based on rapid access to ultrasound and clinical evaluation on the diagnostic certainty in evaluating patients with newly suspected GCA. Methods We reviewed records from patients referred between November 2019 to July 2020, comparing those seen during 17 weeks ‘pre-COVID’, using the twice weekly service, with patients seen during 17 weeks of a 5 day per week service, termed ‘intra-COVID’. All patients underwent clinical evaluation and an eight-site ultrasound of temporal and axillary arteries. Those with hypoechoic, non-compressible peri-luminal arterial wall-thickening (or halo sign) were considered ultrasound ‘positive’. Results We audited 139 patients with suspected GCA (63 pre-COVID period vs 76 intra-COVID). A clinical diagnosis of GCA was made in 33.3% pre-COVID and 42% intra-COVID (p = 0.289). Ultrasound sensitivity improved from 38.1% to 68.6% (p < 0.0001), whilst specificity remained unchanged (100%, p < 0.0001) for both periods. The proportion of patients seen within seven days of referral improved by 61.6% (p < 0.0001), from 31.7% (pre-COVID) to 93.3% (intra-COVID). The median number of days from referral to assessment fell over four-fold, from 12.5 (interquartile range [IQR]=8.25) to 3 (IQR=3.5) respectively. Median number of days of steroid exposure fell over seven-fold from 15 days (IQR=11.25) pre-COVID to 2 days (IQR=5) intra-COVID. Similar proportions in both periods were empirically commenced on steroids prior to assessment (71% vs 68% respectively, p = 0.700). Mean number of ‘halos’ increased from 0.25 (standard deviation [SD]1.19) pre-COVID to 3.05 (SD = 1.82) intra-COVID. The was no significant difference in the number of GCA visual complications (p = 0.894). Ultrasound positivity was associated with fewer days of steroid exposure: the median number of days on steroids in negative versus positive scans was 16 and 5.5 (pre-COVID); compared to 2.5 and 1 days (intra-COVID). The proportion of patients without GCA who were empirically commenced on steroids reduced from 71.4% to 56.8% (pre-COVID vs intra-COVID, p = 0.158) and median days steroid exposure reduced from 9.5 (IQR=17.5) to 1 (IQR=5), respectively. Conclusion A regular, daily ultrasound service for diagnosing suspected GCA significantly improved diagnostic accuracy and reduced unnecessary steroid exposure in patients who did not have GCA. The sensitivity of ultrasound improved by 30.5% and the delay from steroid exposure to diagnosis was reduced by 76%. An ultrasound-based daily fast-track service significantly improved the diagnostic certainty of suspected GCA, especially during the COVID-19 pandemic when availability of temporal artery biopsy was very limited. Disclosure J.P. White: None. G. Sallemi: None. B. Dhungana: None. S. Dubey: None. R.A. Luqmani: None.

P040 An unusual case of giant cell arteritis

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.

Arteritic anterior ischaemic optic neuropathy: An update.

Ischaemic optic neuropathy (ION) is a major cause of blindness. The clinical approach and management is a matter of debate for the treating neurologist and the ophthalmologist. Of the two broad varieties namely the posterior (PION) and anterior ischaemic optic neuropathy (AION), the arteritic variety of the AION (AAION) is usually due to giant cell arteritis. Giant cell arteritis may only present with visual loss in up to 25% of cases. AAION is a neuro-ophthalmic emergency. Early recognition and differentiation from AAION is imperative in order to prevent further devastating visual loss. A literature search was conducted on pubmed using key words as AAION, arteritic anterior ischaemic optic neuropathy, giant cell arteritis (GCA) and articles from the year 2000 till date were included. In any case very few reviews are available on AAION. The literature search on pubmed provided holistic overview about this clinical significant entity and the same is compiled in this review. Moreover the histopathologic features of a temporal artery biopsy have been described with its microscopic images. In any case reviews are mostly available on the Non arteritic variety of AION. This review recapitulates the diagnostic and management protocol of a patient presenting with AAION.

Analysis of Development Mechanism of Giant Cell Arteritis in Nude Mouse Model through Color Duplex Sonography and Computerized Tomography Nanocontrast Agent.

To explore the application value of color duplex sonography and enhanced computerized tomography (CT) inspection based on a nanocontrast agent in diagnosis and pathogenesis in giant cell arteritis (GCA), the GCA nude mouse model was constructed. In this study, 40 healthy male BalB/c nude mice aged 6-8 weeks were randomly divided into a control group (no model) and an experimental group (model), with 20 mice in each group, and the temporal artery tissue of GCA patients diagnosed as positive by temporal artery biopsy was implanted into nude mice to construct a GCA nude mouse model. Abdominal aortic biopsy and immunohistochemistry were used to verify the success of the GCA nude mouse model. All nude mice were subjected to color duplex sonography and enhanced CT examination based on a nanocontrast agent. At the same time, the basic indicators such as body weight, temperature, white blood cell (WBC), lymphocytes (LYM), hemoglobin (HGB), and platelet (PLT) were measured, and the protein expression levels of interleukin-6 (IL-6) and signal transducer and activator of transcription 3 (STAT3) were detected by immunohistochemistry. The results showed that the temporal artery wall of the nude mice in the experimental group thickened and the lumen was significantly narrowed, indicating that the cell arteritis model of nude mice was successfully constructed; ultrasound examination showed that the right superficial temporal artery vascular cavity narrowed, the blood flow signal changed like a filling defect around the periphery, and there was a low echo halo. CT examination showed that the left superficial temporal artery narrowed, and the inner diameter of the narrow segment of blood vessels changed like a bead. The body weight of nude mice in the experimental group decreased significantly after the modeling was completed (P < 0.05); after modeling, the body temperature of the nude mice in the experimental group increased significantly (P < 0.05); LYM and HGB values of nude mice in the experimental group were significantly lower than those in the control group (P < 0.05); the content of IL-6, STAT3, IL-6, and STAT3 proteins in the arterial tissue of nude mice in the experimental group was lower than that of the control group (P < 0.05), indicating that color duplex sonography and CT contrast agent technology can be used in the diagnosis and development mechanism research of GC.

Ultrasound Can Effectively Replace Temporal Artery Biopsy in the Diagnosis of Giant Cell Arteritis: A Multicentre, Prospective Cohort Study

Background: Temporal artery biopsy (TAB) is considered the diagnostic gold standard for giant cell arteritis (GCA), despite up to 39% of biopsy-negative patients receiving a clinical diagnosis of GCA. This is a prospective, multicentre study assessing the diagnostic utility of ultrasound examination in patients with suspected GCA. Methods: Participants were recruited from three Danish centres among patients with clinically suspected GCA. Temporal (TA), facial (FA), common carotid (CC), and axillary arteries (AA) were examined by ultrasonographers, systematically trained in vascular ultrasound, using appropriate equipment and settings, before TAB. A blinded ultrasound expert evaluated all images. The primary outcome was the proportion of participants with clinically diagnosed GCA confirmed after 6-month follow-up. Ultrasound vasculitis was defined in cranial arteries as increased, hypoechoic intima-media thickness (IMT) and a positive compression sign, in large vessels as homogeneous IMT ≥ 1 mm in the AA and ≥1·5 mm in the CA. Findings: One hundred six patients were included in the intention-to-survey population. TAB was positive in 46 patients (43%), and 64 (60%) patients had a clinically confirmed diagnosis of GCA at 6-month follow-up (TAB sensitivity 74%, 95% CI 62–84%, specificity 100%, 95% CI 92–100%). Cranial artery ultrasound was positive in all TAB-positive patients. The sensitivity and specificity of the ultrasound diagnosis of clinical GCA were 94% (84–98%) and 84% (70–93%). Logistic regression analysis confirmed that ultrasound was the strongest baseline predictor for a clinically confirmed diagnosis of GCA at 6 months (crude: OR 76·6, 21·0–280·0; Adjusted for sex and age OR=141·0, 27·0–743). Interpretation: Vascular ultrasound can effectively replace TAB as a first-line diagnostic modality in patients suspected of having GCA when applied by systematically trained ultrasonographers using appropriate equipment and settings. Funding: None to declare. Declaration of Interest: None to declare. Ethical Approval: Written consent was obtained from all participating patients according to the Declaration of Helsinki. The study was approved by The Regional Committees on Health Research Ethics for Southern Denmark (reference number S-2014003) and the Danish Data Protection Agency (reference number 2008-58-0035).

Práctica clínica en la arteritis de células gigantes a partir de una encuesta a especialistas

BackgroundThe purpose of this study was to learn about the clinical practice of specialists who care for patients with giant cell arteritis, to verify whether they follow the diagnosis and treatment recommendations for this disease, and to identify areas for improvement. MethodsA cross-sectional survey on clinical practice in 2019. The survey was completed by 167 physicians (64% rheumatologists, 27% internal medicine specialists, and 9% other specialists) who attended a course on updating giant cell arteritis treatment. We compared the clinical practice collected in the study with the latest recommendations approved by the European League Against Rheumatism (EULAR). ResultsThe physicians surveyed cared for a median of 10 patients (interquartile range 6-30) with giant cell arteritis during their practice. As a diagnostic method, respondents used temporal artery biopsy (84%), temporal artery ultrasound (61%) or other imaging techniques (37%). As first-line therapy, respondents used high-dose glucocorticoids (at least 40mg of prednisone, or equivalent, per day) (84%), glucocorticoids with methotrexate (7%) and glucocorticoids with tocilizumab (5%). The most frequent drugs used for relapse were methotrexate (37%) and tocilizumab (58%). ConclusionOur results indicate that the medical specialists surveyed follow the recent EULAR recommendations for giant cell arteritis diagnosis and therapy.

Giant Cell Arteritis: A Systematic Review and Meta-Analysis of Test Accuracy and Benefits and Harms of Common Treatments.

This systematic review compares treatment options for patients with giant cell arteritis (GCA) and evaluates the test accuracy of studies used in diagnosing and monitoring GCA. These studies were used to inform evidence‐based recommendations for the American College of Rheumatology (ACR)/Vasculitis Foundation (VF) vasculitis management guidelines. A systematic review and search of articles in English in Ovid Medline, PubMed, Embase, and the Cochrane Library was conducted. Articles were screened for suitability, and studies presenting the highest level of evidence were given preference. Three hundred ninety‐nine full‐text articles addressing GCA questions were reviewed to inform 27 Population, Intervention, Comparison, and Outcome questions. No benefit was found with intravenous glucocorticoids (GCs) compared with high‐dose oral GCs in patients with cranial ischemic symptoms (27.4% vs 12.3%; odds ratio [OR] 2.39 [95% confidence interval (CI) 0.75‐7.62], [very low certainty of evidence]). Weekly tocilizumab with a 26‐week GC taper was superior to a 52‐week GC taper in patients achieving remission (risk ratio 4.00 [95% CI 1.97‐8.12], [low certainty of evidence]). Non‐GC immunosuppressive therapies with GCs compared with GCs alone showed no statistically significant in relapse at 1 year (OR 0.87 [95% CI 0.73‐1.04], [moderate certainty of evidence]) or serious adverse events (OR 0.81 [95% CI 0.54‐1.20]; [moderate certainty of evidence]). Temporal artery biopsy has a sensitivity of 61% (95% CI 38%‐79%) and a specificity of 98% (95% CI 95%‐99%) in patients with a clinical diagnosis of suspected GCA. This comprehensive systematic review synthesizes and evaluates the benefits and harms of different treatment options and the accuracy of commonly used tests for the diagnosis and monitoring of GCA.

Heamatological malignancies in giant cell arteritis: a French population-based study.

An increased risk of haematological malignancies (HM) has been reported in GCA patients. Our study aimed to investigate the incidence and the type of HM occurring in GCA. All patients with GCA and HM living in Côte d'Or (France) were identified by crossing data from the RHEMCO (Registre des Hémopathies Malignes de Côte d'Or) and those having a positive temporal artery biopsy between 1 January 2001 and 31 December 2018. Among 276 biopsy-proven GCA patients, 14 HM were identified in 12 patients (4.3%). In comparison with the general population aged >50 y, the incidence of myeloid HM and myeloproliferative syndromes were increased in GCA patients [standardized incidence ratios (SIR) = 2.71 and 5.16, respectively], with a specific increase in men with GCA (SIR = 4.82 and 9.04, respectively) but not in women. In addition, the study of SIR depending on the chronology between GCA and HM diagnoses suggests that there was an increased risk of developing GCA in men but not in women, after a diagnosis of myeloid HM (SIR = 9.56), especially if it was a MPS (SIR = 17.56). Our study shows a particular epidemiology of HM in GCA patients, which is characterized by an increased incidence of myeloid HM, especially MPS, in male GCA patients. The chronology of the diagnoses of GCA and HM raises the hypothesis that clonal hematopoiesis may be implicated in some cases of GCA.