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

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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OP0063 SINGLE CENTRE EXPERIENCE OF THE CLINICAL SPECTRUM, AETIOLOGIES AND MANAGEMENT OF NON-INFECTIOUS AORTITIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1546 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 34.1-34

Author(s):

R. S. Andev ◽

N. Ahmad ◽

A. Verdiyeva ◽

R. Luqmani ◽

S. Dubey

Keyword(s):

Systemic Inflammatory Response Syndrome ◽

Inflammatory Response ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Systemic Inflammatory Response ◽

Aortic Aneurysms ◽

Large Vessel ◽

Complication Rates ◽

Pet Ct ◽

The Mean

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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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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Clinical presentation and treatment response in patients with polymyalgia rheumatica and giant cell arteritis during a 40-week follow-up

Rheumatology Advances in Practice ◽

10.1093/rap/rkab091 ◽

2021 ◽

Author(s):

Amir Emamifar ◽

Søren Hess ◽

Torkell Ellingsen ◽

Oke Gerke ◽

Ziba Ahangarani Farahani ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Treatment Response ◽

Giant Cell ◽

Clinical Features ◽

Polymyalgia Rheumatica ◽

Fdg Pet ◽

Temporal Artery Biopsy ◽

Pet Ct ◽

Fdg Pet Ct

Abstract Objectives To study the clinical features of polymyalgia rheumatica and/or giant cell arteritis (PMR/GCA) and clinical predictors of treatment response during a 40-week follow-up period. Method Clinical data on 77 patients with newly diagnosed PMR/GCA who were treated by oral glucocorticoids were gathered at baseline and during 40-week follow-up period. A unilateral temporal artery biopsy (TAB) and 18 F-FDG PET/CT were undertaken at diagnosis. In total, each patient was seen at 5 occasions i.e. baseline, weeks 4, 16, 28, and 40. Treatment response was assessed considering clinical evaluations and results of inflammatory markers. Results Of 77 patients (49(63.6%) female, mean age : 71.8 ± 8.0), 64(83.1%) patients had pure PMR, 10(13.0%) concomitant PMR and GCA, and 3(3.9%) pure GCA. The patients reported clinical symptoms except scalp pain and duration of morning stiffness improved significantly at week 4 and remained lower at week 40 compared with the relative frequencies at baseline. Besides, all components of physical examination showed significant improvement and remained lower at week 40 compared with the baseline. 68.7%, 62.9%, 44.1% and 33.3% of the patients had a complete response at weeks 4, 16, 28, and 40, respectively. Several clinical features including female gender, younger age, fewer relapse, and lower level of baseline ESR were significantly associated with a better treatment response. Treatment response during follow-up period was independent of TAB results and FDG uptakes on 18 F-FDG PET/CT at diagnosis. Conclusion Obtaining valid disease specific outcome measures for evaluating treatment efficacy in PMR and GCA, that can be applied universally is clearly an unmet clinical need. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT02985424

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Clinical implication of FDG-PET/CT in monitoring disease activity in large-vessel giant cell arteritis linked with secondary polymyalgia rheumatica

Case Reports in Internal Medicine ◽

10.5430/crim.v1n1p6 ◽

2014 ◽

Vol 1 (1) ◽

pp. 6 ◽

Cited By ~ 2

Author(s):

Yoshinori Taniguchi ◽

Shuichi Nakayama ◽

Yoshio Terada

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Polymyalgia Rheumatica ◽

Fdg Pet ◽

Pelvic Girdle ◽

Large Vessel ◽

Pelvic Girdle Pain ◽

Low Grade ◽

Pet Ct ◽

Fdg Pet Ct

Seventy year-old female presented low-grade fever, neck and pelvic girdle pain, jaw claudication and pulseless without visual disturbance. Laboratory examinations showed that C-reactive protein and erythrocyte sedimentation rate (ESR) were 11 mg/dl and 123 mm/1hr, respectively. FDG-PET/CT findings demonstrated bilateral subclavian, carotid and femoral arteritis and aortitis in addition to bursitis and enthesitis of spinous process and pelvic girdle. We diagnosed as large-vessel giant cell arteritis (GCA) linked with secondary polymyalgia rheumatica (PMR). Glucocorticoid therapy was started, and not only these symptoms and but also abnormal findings of FDG-PET/CT were improved.

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AB0354 FDG-PET-DETECTED LARGE VESSEL VASCULITIS DOES NOT PREDICT DISEASE OUTCOME IN PATIENTS WITH GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.570 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1201.3-1202

Author(s):

E. Hysa ◽

D. Camellino ◽

C. Bernini ◽

E. Gotelli ◽

S. Paolino ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Polymyalgia Rheumatica ◽

Clinical Laboratory ◽

C Reactive Protein ◽

Independent Variables ◽

Reactive Protein ◽

Disease Outcomes ◽

Pet Ct

Background:Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are tightly associated inflammatory conditions of the elderly [1]. Both disorders can exhibit an increased articular and vascular uptake of 18-fluorodeoxyglucose (18-FDG) at positron emission tomography (PET)/computed tomography (CT) scan [2].Objectives:This study evaluated if large-vessel vasculitis (LVV) detected by PET/CT in patients with PMR and/or cranial GCA had a negative prognostic value.Methods:108 patients (35 men and 73 women) with a median age of 74 years (range 50-92 years) were prospectively enrolled in our centre over 4 years. PMR was diagnosed by Bird et al. criteria and GCA by the ACR criteria. Six patients died shortly after the first visit (V0) and six were lost at follow-up. Of the remaining 96 patients, 77 were classified as PMR, 6 as GCA and 13 were affected by both diseases.At V0, patients underwent a clinical, laboratory and PET/CT evaluation, and were stratified according to the presence or not of LVV. Follow-up visits were performed every 6 months for a median of 40 months. Disease outcomes were: prednisone (PDN) use and its cumulative dosage, need of methotrexate (MTX), number of relapses, patients’ death, and PMR disease activity score (PMR-DAS). The independent variables were age, sex, disease duration, fever, C-reactive protein (CRP) concentration, platelet count (PLT), presence of cranial GCA, degree of joint and vascular uptake of FDG, and presence of LVV. The predictive role of LVV was tested by multiple regression.Results:LVV was seen in 47 patients (49 %), 31 with PMR, 6 with GCA and 10 with both diseases. Patients with or without LVV did not significantly differ in terms of demographic and laboratory parameters except for a non-significant higher number of PLT in patients with LVV. Clinical and laboratory parameters at V0, stratified per disease and considered together, did not significantly change between PET+ and PET- patients (table 1). Lastly, none of the independent variables, including LVV, could predict disease outcomes.Conclusion:The presence of a PET-detected LVV at diagnosis does not seem a negative prognostic factor in PMR and GCA. As a consequence, routine investigation by PET/CT of patients with PMR and GCA is not indicated to predict disease outcome.References:[1]Dejaco C, Duftner C, Buttgereit F, Matteson EL, Dasgupta B. The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease. Rheumatology (Oxford). 2017 Apr 1;56(4):506-515. doi: 10.1093/rheumatology/kew273. PMID: 27481272.[2]Blockmans D, Coudyzer W, Vanderschueren S, Stroobants S, Loeckx D, Heye S et al. Relationship between fluorodeoxyglucose uptake in the large vessels and late aortic diameter in giant cell arteritis. Rheumatology (Oxford). 2008 Aug;47(8):1179-84. doi: 10.1093/rheumatology/ken119. Epub 2008 May 31. PMID: 18515868.Table 1.Clinical, laboratory and imaging features between PET+ and PET- patients at V0Features at V0PET+ patientsPET- patientspMorning stiffness (min)30 (0-480)60 (0-360)0.20Haemoglobin (g/dL)12.3±1.512.6±1.50.28Platelets (x 103/mm3)349 (108-643)297(159-571)0.08C-reactive protein (mg/dL)35.5 (3.4-149)36.2 (2-149)0.54Erythrocyte sedimentation rate (mm/h)62.5 (10-120)57.5 (10-120)0.29Total Vascular Score at PET20 (4-41)6 (0-12)0Total Joint Score at PET18 (5-30)18 (5-32)0.77Disclosure of Interests:None declared

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Giant-cell arteritis: concordance study between aortic CT angiography and FDG-PET/CT in detection of large-vessel involvement

European Journal of Nuclear Medicine and Molecular Imaging ◽

10.1007/s00259-017-3774-5 ◽

2017 ◽

Vol 44 (13) ◽

pp. 2274-2279 ◽

Cited By ~ 21

Author(s):

Hubert de Boysson ◽

Anael Dumont ◽

Eric Liozon ◽

Marc Lambert ◽

Jonathan Boutemy ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Ct Angiography ◽

Fdg Pet ◽

Large Vessel ◽

Pet Ct ◽

Fdg Pet Ct ◽

Vessel Involvement

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Large vessel vasculitis

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0458 ◽

2020 ◽

pp. 4546-4556

Author(s):

Raashid Luqmani ◽

Cristina Ponte

Keyword(s):

Middle East ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Takayasu Arteritis ◽

Clinical Manifestations ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Distinctive Features ◽

European Women ◽

Vessel Involvement

Large vessel vasculitis describes a group of primary vasculitides predominantly affecting the aorta and its major branches. The two major subtypes of large vessel vasculitis are giant cell arteritis (GCA) and Takayasu arteritis (TA), which have distinctive features. GCA occurs predominantly in white European women aged above 50 years, while TA affects women below 40 years from the Middle East and Asia. However, there are many similarities between GCA and TA, particularly in pathogenic mechanisms, histopathology, and clinical manifestations related to large vessel involvement. It remains unclear whether GCA and TA are distinct entities or represent different phenotypes of the same disease.

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