scholarly journals AB0485 INVESTIGATION OF PERMANENT ORGAN DAMAGE IN GIANT CELL ARTERITIS: DISEASE FLARES ARE ASSOCIATED WITH INCREASED DAMAGE SCORES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1540.2-1540
Author(s):  
B. Ince ◽  
S. Artan ◽  
Y. Yalçinkaya ◽  
B. Artim-Esen ◽  
A. Gül ◽  
...  

Background:Development of organ damage is a major concern in patients with systemic vasculitis. Treatment may also contribute to this important outcome. Scoring systems has been developed to evaluate organ damage in systemic vasculitis and specifically for large vessel vasculitis (1).Objectives:We aimed to investigate permanent organ damage and determining factors in our giant cell arteritis GCA cohort.Methods:Organ damage detected at the time of diagnosis and / or follow-up and irreversible for at least 3 months in GCA patients followed up between 1998-2018 were recorded by using Vasculitis Damage Index (VDI) and Vascular Vasculitis Damage Index (LVVID) fom patient records of our vasculitis clinic. In the statistical evaluation, chi-square, students t-test and logistic regression analysis were used.Results:Eighty-nine patients (64% women, mean age 67.9 ± 9.1) included in the study, the mean follow-up duration was 61.6 ± 58.6 months. All organ damage findings according to both VDI and LVVID are shown in table-1. In this cohort, cardiovascular damage items and diabetes mellitus were prevalent at baseline. At least one damage item was present in 53 (59,5%) according to VDI; 54 (%60,7) according to LVVID and agreement was high between two damage indices (kappa=0.97). Forty-seven of patients (52%) had a damage item presumably with contribution of GC treatment e.g. locomotor system findings, hypertension, diabetes and cataract; 12 (13,5%) had damage items related to disease (total or partial vision loss, ischemic optical neuropathy). Mean time to diagnosis after initial symptoms was longer in patients with permanent vision loss (10,2±4,3 vs. 5,2±1,2 months p=0.006). The presence of damage was associated with flares in univariate and multivariate analysis (29/54 vs. 2/35 p<0,001 OR=19 %95 GA 4,2– 87,9). All patients who had a flare during the first year (n = 15) developed signs of damage at follow-up. No association was found between the development of organ damage and the age of diagnosis, the time between first complaint and diagnosis, presence of cranial, ophthalmologic findings, PET-CT positivity, cumulative steroid dose, and DMARD use.Conclusion:In our study, permanent organ damage was analysed by using diffrerent indices. In this patient population baseline cardiovascular damage and diabetes mellitus were frequent as expected but information for osteoporosis was lacking. More than half of the patients had damage and significant part of the present items was considered due to corticosteroid treatment. The most common damage item developed was osteoporosis. There was a very good agreement between the two indices, despite few specific items in LVVID. The striking relationship of disease flare with damage and frequency of visual problems despite treatment indicate the necessity of new treatment strategies.References:[1]Kermani, T.A., et al.,Evaluation of damage in giant cell arteritis.Rheumatology (Oxford), 2018.57(2): p. 322-328.Disclosure of Interests: :None declared

2019 ◽  
Vol 11 ◽  
pp. 1759720X1982722 ◽  
Author(s):  
Candice Low ◽  
Richard Conway

Giant cell arteritis (GCA) is the most common form of systemic vasculitis. It is a potentially severe disease with 25% of patients suffering vision loss or stroke. Our treatment paradigm is based on glucocorticoids. Glucocorticoids are required in high doses for prolonged periods and subsequently are associated with a significant amount of treatment-related morbidity. Alternative treatment options are urgently needed to minimize these glucocorticoid adverse events. Many other agents, such as methotrexate and tumour necrosis factor alpha inhibitors have been used in GCA, with limited or no evidence of benefit. Our emerging understanding of the pathogenic processes involved in GCA has led to an increased interest in the use of biologic agents to treat the disease. Two randomized controlled trials have recently reported dramatic effects of the use of the interleukin-6 targeted biologic tocilizumab in GCA, with significant increases in remission rates and decreases in glucocorticoid burden. While encouraging, longer-term and additional outcomes are awaited to clarify the exact positioning of tocilizumab in the treatment approach. Emerging data for other biologic agents, particularly abatacept and ustekinumab, are also encouraging but less well advanced. We are at the dawn of a new era in GCA treatment, but uncertainties and opportunities abound.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Jose R. Zaragoza ◽  
Natalia Vernon ◽  
Gisoo Ghaffari

Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries that mainly affects the external carotid artery. It is a diagnosis of the elderly that typically presents as low-grade fever, temporal tenderness, claudication of the jaw, and in some patients vision loss. In cases where GCA presents with atypical manifestations, the diagnosis may be more difficult, causing a delay in both diagnosis and treatment and ultimately leading to irreversible complications. In this paper, we present an atypical presentation of GCA with symptoms of neck swelling and lingual pain in an elderly female. These symptoms progressed to bilateral necrosis and eventual dislodgement of the tongue. Lingual necrosis is a severe potential complication in GCA. In patients presenting with lingual swelling, pain, and discoloration, GCA should be suspected and prompt therapy should be initiated to avoid irreversible complications.


2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
A. Guida ◽  
A. Tufano ◽  
P. Perna ◽  
P. Moscato ◽  
M. T. De Donato ◽  
...  

Giant cell arteritis is a systemic vasculitis characterized by granulomatous inflammation of the aorta and its main vessels. Cardiovascular risk, both for arterial and venous thromboembolism, is increased in these patients, but the role of thromboprophylaxis is still debated. It should be suspected in elderly patients suffering from sudden onset severe headaches, jaw claudication, and visual disease. Early diagnosis is necessary because prognosis depends on the timeliness of treatment: this kind of arteritis can be complicated by vision loss and cerebrovascular strokes. Corticosteroids remain the cornerstone of the pharmacological treatment of GCA. Aspirin seems to be effective in cardiovascular prevention, while the use of anticoagulant therapy is controversial. Association with other rheumatological disease, particularly with polymyalgia rheumatica is well known, while possible association with antiphospholipid syndrome is not established. Large future trials may provide information about the optimal therapy. Other approaches with new drugs, such as TNF-alpha blockades, Il-6 and IL-1 blockade agents, need to be tested in larger trials.


2016 ◽  
Vol 44 (1) ◽  
pp. 78-83 ◽  
Author(s):  
Mikkel Faurschou ◽  
Magnus G. Ahlström ◽  
Jesper Lindhardsen ◽  
Niels Obel ◽  
Bo Baslund

Objective.Patients with organ- or life-threatening vasculitis receive high cumulative glucocorticoid (GC) doses during their disease course. GC have diabetogenic effects, but the risk of diabetes mellitus (DM) related to vasculitis therapy is not well characterized. We assessed the DM risk among patients diagnosed with giant cell arteritis (GCA) or granulomatosis with polyangiitis (GPA), i.e., patients with relatively common forms of systemic vasculitis.Methods.We used Danish healthcare registries to identify 1682 patients diagnosed with GCA and 342 patients diagnosed with GPA from 1997 to 2015 and to obtain information regarding medication exposures. Each patient with vasculitis was matched with 9 population controls. Date of new-onset DM was defined as date of first claimed prescription for an antidiabetic drug. We used Cox regression analyses to calculate incidence rate ratios (IRR) for DM as a measure of the DM risk among patients relative to population controls. Logistic regression was used to study the association between prednisolone/prednisone (PRED) dose and DM.Results.Median duration of followup was 6.5 years [interquartile range (IQR) 2.6–10.4] in the GCA cohort and 5.8 years (IQR 1.7–10.6) in the GPA cohort. During the first year after diagnosis of vasculitis, the IRR for DM was 7.0 (95% CI 5.2–9.3) among patients with GCA and 10.4 (95% CI 4.4–24) among patients with GPA. IRR for DM were not significantly increased in either cohort during later followup periods. Within the first year, treatment with high cumulative prednisolone/PRED doses was associated with new-onset DM among the patients with vasculitis.Conclusion.Patients diagnosed with GCA or GPA have a markedly increased risk of new-onset DM during early treatment phases.


2019 ◽  
Vol 16 (1) ◽  
pp. 109-114
Author(s):  
V. A. Chernukha ◽  
D. S. Atarschikov ◽  
N. V. Khamnagdaeva ◽  
I. V. Pozharov

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.


Rheumatology ◽  
2021 ◽  
Author(s):  
Verena Schönau ◽  
Jessica Roth ◽  
Koray Tascilar ◽  
Giulia Corte ◽  
Bernhard Manger ◽  
...  

Abstract Objectives Efficacy evaluation of giant cell arteritis (GCA) treatment is primarily based on non-specific symptoms and laboratory markers. We aimed to assess the change in vascular inflammation in patients with large vessel (LV)-GCA under different treatments using [18F]FDG PET/CT. Methods Observational study on patients with new-onset, active LV-GCA starting treatment with either prednisolone monotherapy (PRED) or combination with methotrexate (MTX) or tocilizumab (TOC). All patients underwent baseline and follow-up PET/CT. The aorta and its major branches were assessed using PET vascular activity score (PETVAS) by independent readers. Cumulative glucocorticoid doses and cessation of glucocorticoid treatment were documented in all patients. Results We included 88 LV-GCA patients, 27 were treated with PRED, 42 with MTX, and 19 with TOC. PETVAS decreased from 18.9–8.0 units at follow-up in the overall population (p&lt; 0.001). PETVAS changes were numerically higher in patients receiving MTX (-12.3 units) or TOC (-11.7 units) compared with PRED (-8.7). Mean cumulative prednisolone dosages were 5637, 4418, and 2984 mg in patients treated with PRED, MTX, and TOC (p= 0.002). Risk ratios for glucocorticoid discontinuation at the time of follow-up PET/CT were 6.77 (95%CI 1.01–45.29; p= 0.049) and 16.25 (95%CI 2.60–101.73; p= 0.003) for MTX and TOC users compared with PRED users. Conclusion Treatment of LV-GCA inhibits vascular inflammation in the aorta and its major branches. While similar control of vascular inflammation was achieved with PRED, MTX, and TOC treatments, TOC showed a strong glucocorticoid sparing effect, supporting the concept of initial combination therapy.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 689.1-690
Author(s):  
S. Monti ◽  
L. Dagna ◽  
C. Campochiaro ◽  
A. Tomelleri ◽  
G. Zanframundo ◽  
...  

Background:Giant cell arteritis (GCA) is the most frequent systemic vasculitis after the age of 50 years old. Recent interest in the processes of immune and vascular aging have been proposed as a disease risk factor. Data on the impact of age at diagnosis of GCA on the clinical course of the disease are scarceObjectives:To assess the role of age at diagnosis of GCA on the risk and time to relapseMethods:Centres participating in the Italian Society of Rheumatology Vasculitis Study Group retrospectively enrolled patients with a diagnosis of GCA until December 2019. The cohort was divided in tertiles according to age at diagnosis (≤ 72; 73-79; > 79 years old). Negative binomial regression was used to assess the relapse rate according to age groups, and Cox regression for time to first relapse.Results:Of 720 patients enrolled in 14 Italian reference centres, 711 had complete follow-up data (female 50%; mean age 75±7). Median follow-up duration was 34 months (IQR 16;70). Patients in the older group at diagnosis (> 79 years) had more frequent visual loss compared to the 73-79 and ≤ 72 age groups (31% vs 20% vs 7%; p<0.001), but lower rates of general symptoms (56% vs 70% vs 77%; p<0.001). Large-vessel (LV)-GCA was less frequent in the older group (18% vs 22% vs 43%; p<0.001). At least one relapse occurred in 47% of patients. Median time to relapse was 12 months (IQR 6;23). Age did not influence the rate of relapses [18 per 100 persons/years (95%CI 15;21) vs 19 (95% CI 17;22) vs 19 (95%CI 17;22)], nor the time to first relapse (Figure 1). LV-GCA, presentation with significantly elevated c-reactive protein (> 50 mg/L) and general symptoms were independent predictors of relapse.Conclusion:Age at diagnosis of GCA influenced the clinical presentation and risk of ischaemic complications, but did not affect the relapse rate during follow-up. LV-GCA occurred more frequently in younger patients and was an independent predictor of relapse risk, highlighting the need for a correct characterization of the clinical subtype at the early stages of disease.Disclosure of Interests:Sara Monti: None declared, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Alessandro Tomelleri: None declared, Giovanni Zanframundo: None declared, Catherine Klersy: None declared, Francesco Muratore: None declared, Luigi Boiardi: None declared, Roberto Padoan: None declared, Mara Felicetti: None declared, Franco Schiavon: None declared, Milena Bond: None declared, Alvise Berti: None declared, Roberto Bortolotti: None declared, Carlotta Nannini: None declared, Fabrizio Cantini: None declared, Alessandro Giollo: None declared, Edoardo Conticini: None declared, angelica gattamelata: None declared, Roberta Priori: None declared, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer, Elena Treppo: None declared, Giacomo Emmi: None declared, Martina Finocchi: None declared, Giulia Cassone: None declared, Ariela Hoxha Speakers bureau: Celgene, UCB, Novartis, Sanofi, Werfen, Rosario Foti Consultant of: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Speakers bureau: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Michele Colaci: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Carlo Salvarani: None declared, Carlomaurizio Montecucco: None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 397.1-397
Author(s):  
S. Deshayes ◽  
K. Ly ◽  
V. Rieu ◽  
G. Maigné ◽  
N. M. Silva ◽  
...  

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


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Owen Cronin ◽  
Neil D McKay ◽  
Hannah Preston ◽  
Helen Harris ◽  
Barbara Hauser

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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