scholarly journals Associated factors of poor treatment outcomes in patients with giant cell arteritis: clinical implication of large vessel lesions

2020 ◽  
Author(s):  
Takahiko Sugihara ◽  
Hitoshi Hasegawa ◽  
Haruhito A Uchida ◽  
Hajime Yoshifuji ◽  
Yoshiko Watanabe ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Treatment Outcomes ◽  
Immunosuppressive Therapy ◽  
Giant Cell ◽  
Clinical Remission ◽  
Associated Factors ◽  
Signs And Symptoms ◽  
Large Vessel ◽  
Newly Diagnosed ◽  
Poor Treatment

Abstract Background: Relapses frequently occur in giant cell arteritis (GCA) patients treated with conventional immunosuppressive therapy, and identification of associated factors with poor treatment outcomes is relevant to adjust treatment appropriately. Methods: We enrolled 139 newly-diagnosed GCA patients treated with glucocorticoids between 2007 and 2014 in a retrospective, multi-center registry. Patients were diagnosed as having GCA with temporal artery biopsy, large-vessel lesions (LVLs) detected by imaging, 1990 American College of Rheumatology classification criteria, or combination of these. Poor treatment outcomes (non-achievement of clinical remission by week 24 or relapse during 52 weeks) were evaluated. Clinical remission was defined as absence of clinical signs and symptoms in cranial and large-vessel areas, polymyalgia rheumatica (PMR), and elevation of C-reactive protein (CRP) levels. A patient was determined to have relapse if he/she had either one of the signs and symptoms that newly appeared or worsened after achieving clinical remission. Re-elevation of CRP without clinical manifestations was considered as relapse if other causes such as infection were excluded and the treatment was intensified. Associated factors with poor treatment outcomes were analyzed by using the Cox proportional hazard model.Results: Cranial lesions, PMR, and LVLs were detected in 77.7%, 41.7%, and 52.5% of the enrolled patients, respectively. Treatment outcomes were evaluated in 119 newly-diagnosed patients who were observed for 24 weeks or longer. The mean initial dose of prednisolone was 0.76 mg/kg/day, and 29.4% received any concomitant immunosuppressive drugs at baseline. Overall, 41 (34.5%) of the 119 patients had poor treatment outcomes; 13 did not achieve clinical remission by week 24, and 28 had relapse after achieving clinical remission. Cumulative rates of the events of poor treatment outcomes in patients with and without LVLs were 47.5 % and 17.7%, respectively. A multivariable model showed the presence of LVLs at baseline was significantly associated with poor treatment outcomes (adjusted hazard ratio [HR] 3.54, 95% CI 1.52-8.24, p=0.003). Cranial lesions and PMR did not increase the risk of poor treatment outcomes. Conclusion: Presence of LVLs detected by imaging at baseline was an associated factor for poor treatment outcomes in patients given conventional immunosuppressive therapy without biologics.

scholarly journals Associated factors of poor treatment outcomes in patients with giant cell arteritis: clinical implication of large vessel lesions

2020 ◽  
Author(s):  
Takahiko Sugihara ◽  
Hitoshi Hasegawa ◽  
Haruhito A Uchida ◽  
Hajime Yoshifuji ◽  
Yoshiko Watanabe ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Treatment Outcomes ◽  
Giant Cell ◽  
Clinical Remission ◽  
Associated Factors ◽  
Signs And Symptoms ◽  
Classification Criteria ◽  
Large Vessel ◽  
Newly Diagnosed ◽  
Poor Treatment

Abstract Background Relapses frequently occur in giant cell arteritis (GCA) patients treated with conventional immunosuppressive therapy, and identification of associated factors with poor treatment outcomes is relevant to adjust treatment appropriately. Methods We enrolled 139 newly-diagnosed GCA patients treated with glucocorticoids between 2007 and 2014 in a retrospective, multi-center registry. Patients were diagnosed with temporal artery biopsy, 1990 American College of Rheumatology classification criteria, or large-vessel lesions (LVLs) detected by imaging based on the modified classification criteria. Poor treatment outcomes (non-achievement of clinical remission by week 24 or relapse during 52 weeks) were evaluated. Clinical remission was defined as absence of clinical signs and symptoms in cranial and large-vessel areas, polymyalgia rheumatica (PMR), and elevation of C-reactive protein (CRP) levels. A patient was determined to have relapse if he/she had either one of the signs and symptoms that newly appeared or worsened after achieving clinical remission. Re-elevation of CRP without clinical manifestations was considered as relapse if other causes such as infection were excluded and the treatment was intensified. Associated factors with poor treatment outcomes were analyzed by using the Cox proportional hazard model. Results Cranial lesions, PMR, and LVLs were detected in 77.7%, 41.7%, and 52.5% of the enrolled patients, respectively. Treatment outcomes were evaluated in 119 newly-diagnosed patients who were observed for more than 24 weeks. The mean initial dose of prednisolone was 0.76 mg/kg/day, and 29.4% received any concomitant immunosuppressive drugs at baseline. Overall, 41 (34.5%) of the 119 patients had poor treatment outcomes; 13 did not achieve clinical remission by week 24, and 28 had relapse after achieving clinical remission. Cumulative rates of the events of poor treatment outcomes in patients with and without LVLs were 47.5% and 17.7%, respectively. A multivariable model showed the presence of LVLs at baseline was significantly associated with poor treatment outcomes (adjusted hazard ratio [HR] 3.54, 95% CI 1.52-8.24, p=0.003). Cranial lesions and PMR did not increase the risk of poor treatment outcomes. Conclusion Initial treatment intensity in treatment algorithm of GCA could be determined based upon the presence or absence of LVLs detected by imaging at baseline.

scholarly journals P191 Real life experience of tocilizumab for the management of giant cell arteritis in Fife, Scotland

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Paul Allcoat ◽  
Stephanie Hart ◽  
Sarah J Hailwood ◽  
John S McLaren
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Past History ◽  
Real Life ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Service Development ◽  
High Dose ◽  
Nurse Specialist ◽  
Newly Diagnosed

Abstract Background Tocilizumab was accepted by the Scottish Medicines Consortium for use in the management of Giant Cell Arteritis (GCA) on 10.09.18. The Fife Rheumatic Diseases Unit is the first Rheumatology unit in Scotland to introduce a structured process for the management of GCA patients with tocilizumab. We describe here our experience of this new service for NHS Fife. Methods All GCA patients considered for tocilizumab are discussed at our weekly Medication Review Meeting by the Consultant Rheumatologists (JSM & SJH) and Rheumatology Pharmacist (SH). By consensus decision, GCA patients deemed appropriate for tocilizumab treatment are then referred to the Vasculitis Nurse Specialist (VNS; PA) for counselling. We have prospectively recorded data on all GCA patients counselled on tocilizumab from 08.11.18 onwards. Patients are followed up closely in a structured manner by the VNS on a fortnightly basis, with both clinical and laboratory assessment at each visit. The VNS ensures that GCA patients follow a rapid steroid taper in line with that used in the GiACTA Trial. Patients also have laboratory assessments performed between each VNS visit at their GP's surgery (to monitor the ESR) to assist in decision making in view of the rapid steroid taper. The VNS also records the cumulative steroid dose along with drug-related side effects (both from steroids and tocilizumab). Results 14 GCA patients have been counselled and commenced on tocilizumab between 08.11.18 and 18.09.19. We have another 2 GCA patients awaiting counselling and due to start tocilizumab soon. Twelve patients have newly diagnosed GCA (5 with unilateral visual loss), 5 with Large vessel vasculitis and 3 patients have refractory GCA. Of the newly diagnosed patients, the reasons for initiating tocilizumab were as follows: poor control of diabetes mellitus on high dose steroids; large vessel vasculitis confirmed on PET scan; previous long-term steroid exposure due to past history of polymyositis; high osteoporotic risk. Conclusion We have successfully introduced a structured process for the use of tocilizumab in Fife GCA patients via a VNS-led GCA clinic. This ensures robust clinical governance around the issues of safety, efficacy and cost. In addition, by collecting real life prospective data, we will add to the existing information available regarding use of this new therapeutic advance for GCA management in routine clinical practice. This initiative is the first of its kind in Scotland and a new service development for NHS Fife. Disclosures P. Allcoat None. S. Hart None. S.J. Hailwood None. J.S. McLaren Honoraria; J.S.M. has received Speaker fees from Roche.

Patterns of extracranial involvement in newly diagnosed giant cell arteritis assessed by physical examination, colour coded duplex sonography and FDG-PET

VASA ◽  
2011 ◽  
Vol 40 (3) ◽  
pp. 219-227 ◽  
Author(s):  
Förster ◽  
Tato ◽  
Weiss ◽  
Czihal ◽  
Rominger ◽  
...  
Keyword(s):  
Physical Examination ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Clinical Diagnosis ◽  
Lower Limb ◽  
Fdg Pet ◽  
Duplex Sonography ◽  
Large Vessel ◽  
Newly Diagnosed ◽  
Vessel Involvement

Background: The clinical spectrum of giant cell arteritis (GCA) varies from classical temporal arteritis (TA) to generalized large vessel GCA (LV-GCA) and fever of unknown origin (FUO). Extent and distribution of extracranial involvement in these different presentations of GCA is not well known, and its detection may depend on the choice of vascular imaging. Patients and methods: In 24 patients with newly diagnosed GCA we systematically evaluated the presence and distribution of extracranial involvement by physical examination, duplex sonography (DS), and FDG-PET. Analysis of FDG-PET results was performed in comparison with 18 age-matched control-subjects scanned for oncological indications. Results: Initial clinical diagnosis was TA in 11 patients, LV-GCA in 8 patients, and FUO in 5 patients. Clinically detectable arterial obstruction was present in 2 patients (18 %) with TA (only upper extremity), all patients with LV-GCA (upper and lower extremities) and no patient with FUO. Upper and/or lower limb large vessel vasculitis was detectable by DS in 45 % of the patients with TA and in 100 % of the patients with LV-GCA or FUO. FDG-PET confirmed upper extremity involvement in all affected patients, but had a very low specificity for lower limb involvement due to concomitant arteriosclerosis in these elderly patients. Aortitis was detectable by FDG-PET in 27 % of patients with TA and 75 - 80 % of patients with LV-GCA or FUO. Conclusions: The combination of thorough clinical examination and DS is able to detect symptomatic as well as asymptomatic large vessel involvement in a large proportion of patients with newly diagnosed GCA. Distribution and manifestation of large vessel involvement differs between classical TA and LVGCA or FUO. FDG-PET provided only limited additional information and did not change the clinical diagnosis in any patient.

Large vessel involvement in biopsy-proven giant cell arteritis: prospective study in 40 newly diagnosed patients using CT angiography

2012 ◽  
Vol 71 (7) ◽  
pp. 1170-1176 ◽  
Author(s):  
Sergio Prieto-González ◽  
Pedro Arguis ◽  
Ana García-Martínez ◽  
Georgina Espígol-Frigolé ◽  
Itziar Tavera-Bahillo ◽  
...  
Keyword(s):  
Prospective Study ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Ct Angiography ◽  
Large Vessel ◽  
Newly Diagnosed ◽  
Vessel Involvement

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

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

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

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

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

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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