Giant cells in temporal artery biopsies are associated with a reduced risk of large vessel involvement in patients with giant cell arteritis : a retrospective cohort study

Abstract Background: Giant cell arteritis (GCA) is a systemic disease with extensive vascular involvement. There is limited and conflicting information on the relation between patient characteristics at diagnosis and future disease phenotypes. We aimed to investigate predictors of time dependent large vessel involvement (LVI) in a population-based cohort of patients with GCA. Methods: GCA patients with positive temporal artery biopsies (TAB) between 1997and 2010 were identified through a regional pathology register. A structured review of histopathology reports and relevant imaging studies was performed. Cases with LVI through July 2016 were identified. Patients were followed to first LVI, death, migration from the area or July 29, 2016. Event free survival by clinical and histopathologic features was estimated using the Kaplan-Meier method. Potential predictors of LVI were examined using Cox regression models.Results: A total of 274 patients were included. The mean age at GCA diagnosis was 75.7 years. Fifty-one patients (19 %) had documented LVI during the follow-up, corresponding to an incidence rate of 2.4/100 person-years. The median time from GCA diagnosis to the diagnosis of LVI was 4.5 years (interquartile range 0.6-7.4). Thirty-four patients had aortic involvement (67% of those with LVI; 12% of all GCA cases). Survival free of LVI was longer in patients with giant cells in the TAB (75th percentile 14.0 vs 6.7 years; p=0.014). In age-adjusted analysis, the presence of giant cells in the TAB was associated with reduced risk of LVI (hazard ratio 0.48; 95 % confidence interval 0.27-0.86). Conclusions: The negative association with giant cells in the TAB suggests that patients with LVI constitute a subset of GCA with particular disease mechanisms. This underlines the heterogeneity of GCA, which should be further explored in prospective studies.

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AB0501 THREE CASES OF VERTEBRAL ARTERITIS IDENTIFIED ON FDG-PET IN PATIENTS WITH SUSPECTED GCA AT UNIVERSITY COLLEGE LONDON HOSPITAL (UCLH)

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.410 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1549.2-1549

Author(s):

D. Ludwig ◽

M. Naja ◽

S. Voo ◽

V. Morris

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Pet Imaging ◽

Fdg Pet ◽

Immunosuppressive Treatment ◽

Temporal Artery ◽

Large Vessel ◽

Diagnostic Tools ◽

History Of ◽

Vessel Involvement

Background:Giant cell arteritis (GCA) may affect both cranial and extra-cranial vessels; where the latter occurs, it can be termed large-vessel GCA (LV-GCA). Large vessel involvement is common: histological evidence has been seen in 80% of autopsies of patients with known GCA, and imaging studies suggest large vessel involvement in over 80%1. LV-GCA is important to diagnose due to the risks of vascular complications such as occlusion and ischaemic stroke. The clinical diagnosis can be challenging, and the American College of Rheumatology (ACR) GCA classification criteria often underperform in cases of LV-GCA1. F-fluorodeoxyglucose positron emission tomography (FDG-PET) has been found to be useful in the detection of extra-cranial involvement to support the diagnosis of LV-GCA.2Objectives:To appreciate the variability in presentation of cases of LV-GCA, and to further characterise a subgroup of patients with vertebral arteritis.To explore the use of FDG-PET imaging in GCA patients in addition to or in place of traditional diagnostic tools (temporal artery ultrasound / biopsy).Methods:Through evaluation of the new GCA fast-track pathway implemented at UCLH, a subgroup of patients diagnosed with vertebral arteritis was identified. The history and presentation of these patients were analysed.Results:Three patients were diagnosed with vertebral arteritis. All three were male, Caucasian and aged over 70. All were investigated for GCA due to a history of severe headache (frontal in one, occipital in one, bi-temporal in one) with associated red flag symptoms. Two had a history of jaw claudication and visual disturbances (unilateral visual loss in one, transient diplopia in the other). Both of these patients had positive temporal artery biopsies. The third patient had no ischaemic symptoms but a strong history of prominent polymyalgic features and a positive temporal artery ultrasound. Inflammatory markers were raised in two, and normal in one, of the patients. Only one had systemic symptoms (weight loss). All three proceeded to FDG-PET scans which showed vertebral arteritis and were commenced on immunosuppressive treatment.Conclusion:The cases discussed illustrate the heterogeneity of the presentation of LV-GCA, and the diagnostic challenge this poses. FDG-PET imaging is useful in confirming extra-cranial involvement and therefore guiding treatment.References:[1]Large-vessel giant cell arteritis: diagnosis, monitoring and management.Matthew J Koster, Eric L Matteson, Kenneth J Warrington.2018, Rheumatology, Vol. 57, pp. 32-42.[2]EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice.Dejaco C, Ramiro S, Duftner C, et al.2018, Annals of the Rheumatic Diseases, Vol. 77, pp. 636-643.Disclosure of Interests: :None declared

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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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Large-vessel Involvement and Varicella Zoster Virus Vasculopathy in Giant Cell Arteritis–related Stroke: Something to Keep an Eye On

The Journal of Rheumatology ◽

10.3899/jrheum.170317 ◽

2017 ◽

Vol 44 (10) ◽

pp. 1566-1566 ◽

Cited By ~ 1

Author(s):

GIANFRANCO VITIELLO ◽

DANIELE CAMMELLI

Keyword(s):

Varicella Zoster Virus ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Large Vessel ◽

Varicella Zoster ◽

Vessel Involvement

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JAK Inhibitor Effectiveness in Giant-Cell Arteritis With Large-Vessel Involvement Assessed by 18F-FDG PET-CT

Clinical Nuclear Medicine ◽

10.1097/rlu.0000000000003913 ◽

2021 ◽

Vol Publish Ahead of Print ◽

Author(s):

Kevin Prigent ◽

Achille Aouba ◽

Nicolas Aide ◽

Hubert de Boysson

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Fdg Pet ◽

Large Vessel ◽

Jak Inhibitor ◽

Pet Ct ◽

18F Fdg ◽

Fdg Pet Ct ◽

Vessel Involvement

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An unusual presentation of giant cell arteritis

VASA ◽

10.1024/0301-1526.34.2.128 ◽

2005 ◽

Vol 34 (2) ◽

pp. 128-130 ◽

Cited By ~ 8

Author(s):

Simon ◽

Fritz ◽

Amann-Vesti ◽

Schenk Romer ◽

Fischer ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Nasal Septum ◽

Rare Complication ◽

Temporal Artery ◽

Temporal Artery Biopsy ◽

Elderly Persons ◽

First Case ◽

Scalp Necrosis ◽

Vessel Involvement

A 77-year-old-man with giant cell arteritis who developed bitemporal scalp ulcerations is described. Since 1946 when Cooke et al. reported the first case of scalp necrosis there were approximately 55 cases published. Scalp ulceration is a rare complication of giant cell arteritis and occurs mainly in elderly persons, particularly women. About half of all patients were presented to dermatologists. Most of the patients (70%) had other serious complications of giant cell arteritis: blindness, gangrene of the tongue and nasal septum necrosis. Seventy percent of the cases were confirmed by a temporal artery biopsy. The necrosis were of varying extent and uni- or bilateral. Although, in most cases necrosis has been located bilaterally as in the presented case. Scalp healing was complete nearly in all patients by conservative treatment within a year. Scalp ulceration is a potentially reversible complication of giant cell arteritis which indicates extensive vessel involvement and adequate coricosteroid therapy is required and essential.

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