Normal inflammatory markers in giant cell arteritis with long-standing cranial and symptomatic large-vessel involvement

2021 ◽  
Vol 14 (6) ◽  
pp. e242602
Author(s):  
Alicia Rodriguez-Pla ◽  
Sailendra G Naidu ◽  
Yasmeen M Butt ◽  
Victor J Davila
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Markers ◽  
Care Physician ◽  
Large Vessel ◽  
High Dose ◽  
Surgical Revascularisation ◽  
History Of ◽  
Surgery Department ◽  
Vessel Involvement

We report the case of a 78-year-old woman who presented with cardiovascular risk factors and a history of an atypical transient ischaemic attack. She was referred by her primary care physician to the vascular surgery department at our institution for evaluation of progressive weakness, fatigue, arm claudication and difficulty assessing the blood pressure in her right arm. She was being considered for surgical revascularisation, but a careful history and review of her imaging studies raised suspicion for vasculitis, despite her normal inflammatory markers. She was eventually diagnosed with biopsy-proven giant cell arteritis with diffuse large-vessel involvement. Her symptoms improved with high-dose glucocorticoids.

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

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

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

Rheumatology ◽  
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.

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

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

scholarly journals Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis

2018 ◽  
pp. bcr-2018-225304
Author(s):  
Gesine Boisch ◽  
Sven Duda ◽  
Christian Hartmann ◽  
Heinrich Weßling
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Arteritis ◽  
Brain Mri ◽  
Neurological Deficits ◽  
High Dose ◽  
Immunoglobulin G4 ◽  
Perifocal Oedema ◽  
History Of ◽  
Dose Corticosteroid

We report the rare case of an 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented to our neurological department with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral and postcentral cortex. A leptomeningeal biopsy was performed. An autoimmune-mediated immunoglobulin G4-associated hypertrophic pachymeningoencephalitis was diagnosed. The patient received a high-dose corticosteroid therapy and his symptoms gradually improved. Our results suggest that hypertrophic pachymeningoencephalitis may occur as a complication of giant cell arteritis and may cause central neurological deficits by cerebral perifocal oedema.

Progressive and Fatal Brainstem Stroke in Systemic Giant Cell Arteritis

2021 ◽  
pp. 10.1212/CPJ.0000000000001083
Author(s):  
Valentina Poretto ◽  
Silvio Piffer ◽  
Valeria Bignamini ◽  
Enzo Tranquillini ◽  
Davide Donner ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Facial Artery ◽  
High Dose ◽  
Temporal Artery Biopsy ◽  
Halo Sign ◽  
Pet Ct ◽  
Brainstem Stroke ◽  
History Of

A 74-year-old woman presented with acute worsening of six-months long history of vertigo and postural instability, with MRI evidence of cerebellar and brainstem acute infarcts. Extensive neurovascular assessment revealed a severe vascular damage with multiple stenoses and occlusions along vertebrobasilar axis (figure 1). Duplex ultrasonography showed hypoechoic halo sign along facial artery, while PET-CT highlighted increased [18F]-FDG uptake along vertebral and other larger arteries, thus allowing a diagnosis of giant cell arteritis (figure 2).1,2 Despite prompt treatment with high-dose steroids and tocilizumab, which probably made uninformative a subsequent temporal artery biopsy (figure 2), patient died of reported disability after strokes.

Giant-Cell Arteritis: Do We Treat Patients with Large-Vessel Involvement Differently?

2017 ◽  
Vol 130 (8) ◽  
pp. 992-995 ◽  
Author(s):  
Hubert de Boysson ◽  
Eric Liozon ◽  
Marc Lambert ◽  
Anael Dumont ◽  
Jonathan Boutemy ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Large Vessel ◽  
Vessel Involvement
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