COVID-19 vaccination and large-vessel giant cell arteritis

Background:Aortitis, a rare form of large vessel vasculitis, may occur in the context of a primary systemic vasculitis, as a part of systemic autoimmune disease or in isolation. The evidence and guidelines to diagnose, manage and monitor aortitis remain limited. However, PET CT and vascular MRI scans have facilitated our ability to make the diagnosis more readily. The optimal management strategy and complication rates remain uncertain.Objectives:Our aim was to explore the clinical, laboratory and radiological features of aortitis. We sought to review the management and complications of this illness by collecting detailed information on the outcomes and treatments used, including disease modifying agents (DMARDs) and biologics.Methods:Patients diagnosed with aortitis since 2006 that had been managed in a single tertiary centre were identified using the Rheumatology Assessment Database Innovation in Oxford (RHADIO). Their medical notes were retrospectively reviewed using a local electronic patient record system and the following information was obtained: demographics, underlying risk factors, imaging and laboratory results (including biopsy reports if available), management and outcome.Results:We identified 155 patients who met the inclusion criteria. There was a female preponderance of 57.4% (n=89). At the time of diagnosis, the average age was 69 (range 30-92) and the mean symptomatology length prior to diagnosis was 12 months (range 0-120). The majority of patients (60.4%, n=94) had aortitis secondary to giant cell arteritis (GCA), isolated aortitis was identified in 29.7% (n=46) and IgG4-related disease aortitis was uncommon (2.6%, n=4). Those with cranial GCA-like symptoms were diagnosed on average 3.9 months before those who presented differently (10.1 months versus 14.0 months).Common presentations comprised: systemic inflammatory response syndrome (49.0%, n=76), cranial GCA-like symptoms (26.5%, n=41) and unexplained weight loss (24.5%, n=38). Importantly, 18.7% (n=29) of patients presented with ischaemic symptoms that included angina, TIAs/strokes and claudication. Aortic dissection was the primary presentation for 6.5% (n=10) of patients.At presentation, the mean CRP was 84 mg/L (range 1-249) and the ESR was 72 mm/hr (range 2-164). Most (73.5%, n=114) had diagnostic PET CT changes. For those patients with GCA, diagnostic ultrasound changes were seen in 27.7% (n=26).Nearly all were treated with prednisolone (92.3%, n=143) and all but 8 (5.1%) received a DMARD at some point. Methotrexate was the most commonly used DMARD (93.9%, n=138), followed by leflunomide (22.3%, n=35) and azathioprine (19.1%, n=28). Cyclophosphamide was used in 23.8% of patients (n=38) and 15 patients (9.7%) received tocilizumab.Around a third (34.1% n=53/155) had received at least two DMARDs during their treatment course. On average, patients required 3.46 drugs to manage their aortitis. Those who relapsed (43.2%, n=67) were more likely to have GCA (65.7%, n=44).Vascular sequelae were present in 37.4% (n=58). The most common complications were ischaemic in nature with stroke/TIA and claudication reported in 16.8% (n=26). Aortic aneurysms were recorded in 11.6% (n=18) of cases and 5.1% (n=8) developed dissections despite being on treatment for their aortitis. One patient developed renal infarcts and ischaemic bowel leading to intestinal failure because of florid vasculitis.Conclusion:Aortitis has a varied presentation with systemic inflammatory response syndrome being the most common. Delayed diagnosis remains a problem and especially for those with non-GCA related aortitis, which is likely to contribute to the risk of subsequent vascular complications. Vascular events including dissection are common, many of which could be preventable, emphasising the importance of early diagnosis and good disease control.References:[1]Koster M et al. Large-vessel giant cell arteritis: diagnosis, monitoring and management. Rheumatology [Internet]. 2018 Feb 1;57(suppl_2):ii32–42. Available from: https://doi.org/10.1093/rheumatology/kex424Disclosure of Interests:None declared

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POS0338 STEROID-SPARING EFFECT OF ANAKINRA IN GIANT-CELL ARTERITIS: A CASE SERIES WITH CLINICAL, BIOLOGICAL AND ICONOGRAPHIC LONG-TERM ASSESSMENTS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1623 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 397.1-397

Author(s):

S. Deshayes ◽

K. Ly ◽

V. Rieu ◽

G. Maigné ◽

N. M. Silva ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Interleukin 1 ◽

Oral Prednisone ◽

Large Vessel ◽

Daily Dose ◽

Sparing Effect ◽

Steroid Sparing

Background:The treatment of giant cell arteritis (GCA) relies on corticosteroids but is burdened by a high rate of relapses and adverse effects. Anti-interleukin-6 treatments show a clear benefit with a significant steroid-sparing effect, but late relapses occur after treatment discontinuation. In addition to interleukin-6, interleukin-1 also appears to play a significant role in GCA pathophysiology.Objectives:We report herein the efficacy of anakinra, an interleukin-1 receptor antagonist, in 6 GCA patients exhibiting corticosteroid dependence or resistance, specifically analyzing the outcome of aortitis in 4 of them, and including the long-term follow-up of 2 previously described patients (1).Methods:This retrospective study analyzed the cases of all GCA patients treated with anakinra from the French Study Group for Large Vessel Vasculitis.Patients had to satisfy the following two criteria to be enrolled in this retrospective study. First, their diagnosis of GCA should be based on the fulfillment of at least 3 criteria of the American College of Rheumatology (ACR) for GCA or on the satisfaction of 2 of these criteria along with the demonstration of LVI on imaging. Second, patients should have received anakinra because of corticosteroid dependence or resistance.Corticosteroid dependence was defined as ≥2 relapses or the combination of 2 of the following criteria: a daily dose of oral prednisone >20 mg/day (or 0.3 mg/kg) at 6 months; a daily dose of oral prednisone >10 mg/day (or 0.2 mg/kg) at 12 months; and/or a treatment maintained >24 months because of a relapsing disease course. Corticosteroid resistance was defined as persistent increased inflammatory parameters at month 3 despite a steroid dosage over 0.5 mg/kg.Results:After a median duration of anakinra therapy of 19 [18–32] months, all 6 patients exhibited complete clinical and biological remission. Among the 4 patients with large-vessel involvement, 2 had a disappearance of aortitis under anakinra, and 2 showed a decrease in vascular uptake. After a median follow-up of 56 [48–63] months, corticosteroids were discontinued in 4 patients, and corticosteroid dosage could be decreased to 5 mg/day in 2 patients. One patient relapsed 13 months after anakinra introduction in the context of increasing the daily anakinra injection interval to every 48 hours. Three patients experienced transient injection-site reactions, and 1 patient had pneumonia.Figure 1.Steroid dosages before and after the introduction of anakinra in 6 patients with giant-cell arteritis and corticosteroid dependence or resistance. The black arrow indicates the time of anakinra introduction.Conclusion:In this short series, anakinra appears to be an efficient and safe steroid-sparing agent in refractory GCA, with a possible beneficial effect on large-vessel involvement.References:[1]Ly K-H, Stirnemann J, Liozon E, Michel M, Fain O, Fauchais A-L. Interleukin-1 blockade in refractory giant cell arteritis. Joint Bone Spine 2014;81:76–8.Disclosure of Interests:Samuel Deshayes: None declared, Kim LY: None declared, Virginie Rieu: None declared, Gwénola Maigné: None declared, Nicolas Martin Silva: None declared, Alain Manrique: None declared, Jacques Monteil: None declared, Hubert de Boysson Speakers bureau: Roche-Chugai, Grant/research support from: Roche-Chugai, Achille Aouba Grant/research support from: SOBI

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POS0803 TRENDS IN THE INCIDENCE OF GIANT CELL ARTERITIS ACROSS SEVEN DECADES AND CHANGES IN DIAGNOSTIC MODALITIES: A POPULATION-BASED STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1415 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 654.1-654

Author(s):

T. Garvey ◽

C. S. Crowson ◽

M. Koster ◽

K. J. Warrington

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Population Based ◽

Incidence Rates ◽

Large Vessel ◽

Vessel Imaging ◽

Group 3 ◽

Group 2 ◽

Diagnostic Modalities ◽

Incident Cases

Background:Diagnostic methods for giant cell arteritis (GCA) have evolved over recent decades, and large vessel imaging plays an increasing role in disease detection.Objectives:This study aims to estimate the incidence of GCA over the past 10 years in a population and compare it to preceding incidence estimates. It also explores trends in the diagnostic modalities used to identify GCA.Methods:A pre-existing population-based cohort of patients diagnosed with GCA between 1950 and 2009 was extended with incident cases from 2010 to 2019. The diagnosis of GCA was confirmed by review of medical records of patients with ICD9/10 codes for GCA between 1/1/2010 and 12/31/2019. Incident cases that met either one of the following sets of inclusion criteria were added to the cohort: one, American College of Rheumatology 1990 GCA classification criteria; or two, patients aged ≥50 years with elevation of erythrocyte sedimentation rate or C-reactive protein and radiographic evidence of large vessel vasculitis attributed to GCA. Incident cases were classified into one of three groups: group 1, temporal artery biopsy (TAB) positive; group 2, TAB negative or not done with positive large-vessel imaging; or group 3, clinical diagnosis of GCA.Results:The study cohort included 305 patients diagnosed with GCA from 1950 until 2019. Fifty-five incident cases were diagnosed between 2010 and 2019; 37 females (67%) and 18 males (33%). The age and sex adjusted incidence rates (95% CI) per 100,000 between 2010 and 2019 for females, males, and the total population were 13.0 (8.8, 17.3), 8.6 (4.6, 12.7), and 10.8 (8.0, 13.7), respectively. The corresponding incidence rates from 2000-2009 were 28.0 (21.0, 35.1), 10.2 (5.0, 15.5), and 20.5 (15.9, 25.1), respectively. This represents a significant decline in the incidence rates in females (p<0.001) and the total group (p<0.001) between the 2000-2009 and 2010-2019 cohorts but no change in males (p=0.64). Of the 55 patients diagnosed between 2010 and 2019, there were 37 (67%) in group 1, 10 (18%) in group 2, and 8 (15%) in group 3. In contrast, of the 250 patients diagnosed between 1950 and 2009 there were 209 (84%) in group 1, 4 (2%) in group 2, and 37 (15%) in group 3. There was a significant difference between the 1950-2009 and 2010-2019 cohorts in the composition of these groups (p<0.001).Conclusion:In this population-based cohort of patients with GCA diagnosed over a 70-year period, the incidence of GCA has declined in recent years. The total decline is driven by a decline in females but not in males. The reasons for this are unclear but should be followed over time and investigated in other population-based cohorts. There has also been a shift in the diagnostic modalities for GCA. In recent years, there are fewer TAB positive patients, and more patients diagnosed with large vessel imaging. This is the first population-based incidence cohort demonstrating a trend towards increased use of large vessel imaging for the diagnosis of GCA.References:[1]Chandran AK, et al. Incidence of Giant Cell Arteritis in Olmsted County, Minnesota, over a 60-year period 1950-2009. Scand J Rheumatol. 2015;44(3):215-218.[2]Gonzalez-Gay MA, et al. Giant cell arteritis: is the clinical spectrum of the disease changing? BMC Geriatr. 2019; Jul 29;19(1):200.[3]Rubenstein E, et al. Sensitivity of temporal artery biopsy in the diagnosis of giant cell arteritis: a systemic literature review and meta-analysis. Rheumatology (Oxford). 2020 May 1:59(5):1011-1020.Figure 1.Trends in the incidence of GCA in Olmsted County by sex (1950-2019).Acknowledgements:This study was made possible using the resources of the Rochester Epidemiology Project, which is supported by the National Institute on Aging of the National Institutes of Health (NIH) under Award Number R01 AG034676, and CTSA Grant Number UL1 TR000135 from the National Center for Advancing Translational Sciences (NCATS), a component of the NIH. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.Disclosure of Interests:Thomas Garvey: None declared, Cynthia S. Crowson: None declared, Matthew Koster: None declared, Kenneth J Warrington Grant/research support from: Clinical research support from Eli Lilly and Kiniksa

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Comment on: Evaluation of adjunctive mycophenolate for large vessel giant cell arteritis

Rheumatology Advances in Practice ◽

10.1093/rap/rkab011 ◽

2021 ◽

Vol 5 (1) ◽

Author(s):

Rifat Mazumder ◽

Chetan Mukhtyar

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Large Vessel

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P205 The contemporary presentation and management of giant cell arteritis with large vessel vasculitis

Rheumatology ◽

10.1093/rheumatology/keab247.200 ◽

2021 ◽

Vol 60 (Supplement_1) ◽

Author(s):

Owen Cronin ◽

Neil D McKay ◽

Hannah Preston ◽

Helen Harris ◽

Barbara Hauser

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Polymyalgia Rheumatica ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Ct Angiogram ◽

Pet Ct ◽

The Mean ◽

Vessel Involvement

Abstract Background/Aims Giant cell arteritis with large vessel vasculitis (LV-GCA) represents a distinct, less researched sub-category of giant cell arteritis (GCA). In comparison to cranial GCA, the patient’s diagnostic pathway is less well described and it is thought that LV-GCA is underdiagnosed, including in patients with polymyalgia rheumatica and cranial-GCA. Advances in imaging (e.g. PET-CT) and treatment (tocilizumab), have provided additional options in the diagnosis and management of LV-GCA. The aim was to describe the contemporary clinical journey for patients diagnosed with LV-GCA. Methods The electronic patient health record system in NHS Lothian (TrakCare) was used to collect relevant data. Patients with imaging-confirmed large vessel vasculitis, diagnosed with GCA after 1 January 2017 were included. Follow-up was until August 2020. Results Eighteen patients with LV-GCA were included. The mean age was 65 years and 66.7% were female. Two patients had known cranial-GCA but 89% of patients were diagnosed exclusively with large vessel involvement. The most common symptoms were malaise (55%), weight loss (55%), polymyalgia rheumatica (55%) and limb claudication (44%). Pyrexia of unknown origin was a feature in only 17% of patients. Two patients were asymptomatic and were investigated on the basis of raised inflammatory markers. Mean CRP at baseline was 99mg/L and ESR 85mm/hour. The mean time from symptom-onset to diagnosis was 6.8 months (range 1 to 15 months). Sixteen patients (89%) were reviewed by at least one other secondary care specialist. One third of patients were referred from General Medicine followed by Vascular Surgery (16%) and General Practice (16%). 7/18 patients were inpatients at the time of referral. 56% of patients required two modalities of imaging to confirm large vessel involvement. The most commonly used imaging techniques (in descending order) were CT-Chest/Abdomen/Pelvis, CT-angiogram, PET-CT and Vascular Ultrasound. 50% of patients underwent follow-up imaging, most commonly MR- or CT-angiography. Mean follow-up was for 1.6 years. The mean prednisolone dose at 3 months (n = 18) was 24mg daily and 8mg at 12 months (n = 12). 28% of patients relapsed during the follow-up period at 4, 5, 8, 9 and 24 months post-diagnosis. 7/18 patients were commenced on methotrexate for steroid-side effects or for relapse. 8/18 received subcutaneous tocilizumab in combination with methotrexate in two cases. Three patients were started on azathioprine but only one continued. Conclusion In modern-day clinical practice, patients with LV-GCA experience a longer time to diagnosis than those with cranial symptoms. Patients with LV-GCA can experience an array of constitutional symptoms. Frequently, more than one imaging modality is required to confirm LV-GCA and the majority of patients will have seen other hospital specialists or have been admitted to hospital before diagnosis. Methotrexate and tocilizumab are the most frequently-used and effective steroid-adjunct in this single-centre cohort. Disclosure O. Cronin: None. N.D. McKay: Consultancies; Gilead. Other; Has received support for conference attendance from Pfizer and Gilead, Has received educational support from UCB, Gilead, Celgene, Biogen, Sanofi, Abbvie, Novartis, Pfizer. H. Preston: None. H. Harris: None. B. Hauser: None.

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FRI0197 THE DIAGNOSTIC ACCURACY OF VASCULAR ULTRASOUND IN PATIENTS SUSPECTED OF GCA: A DANISH MULTICENTRE PROSPECTIVE COHORT STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3953 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 682.1-682

Author(s):

S. Chrysidis ◽

U. Møller Døhn ◽

L. Terslev ◽

U. Fredberg ◽

T. Lorenzen ◽

...

Keyword(s):

Multicentre Study ◽

Diagnostic Accuracy ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Clinical Diagnosis ◽

Axillary Artery ◽

Laboratory Data ◽

Temporal Artery ◽

Large Vessel ◽

The Us

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

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