scholarly journals Mortality in Patients with Biopsy-proven Giant Cell Arteritis: A South Australian Population-based Study

2011 ◽  
Vol 38 (10) ◽  
pp. 2215-2217 ◽  
Author(s):  
JEM NINAN ◽  
ANH-MINH NGUYEN ◽  
ANTONIA COLE ◽  
MAUREEN RISCHMUELLER ◽  
THOMAS DODD ◽  
...  

Objective.To compare mortality rates and cause of death in patients with biopsy-proven giant cell arteritis (GCA) with those in the general population.Methods.Patients with biopsy-proven GCA were identified from pathology reports of temporal artery biopsies in South Australia, from January 1, 1992, to December 31, 2006. All patients with biopsy-proven GCA were linked to the South Australian Births, Death and Marriage Registry to identify deaths until December 31, 2006. Standardized mortality ratios and relative survival (ratio of observed survival in GCA group to expected survival of general South Australian population, matched by age, sex, and calendar time) were calculated. The cause of death recorded on the death certificate was also documented.Results.There were 225 cases of biopsy-proven GCA (163 women and 62 men). The mean age at diagnosis of GCA was 78.2 years. The mean followup period was 66.2 months (SD 47.1 mo). During the followup period, there were 71 deaths in the GCA group (50 women, 21 men). The standardized mortality ratio was 0.99 (95% CI 0.77–1.25). The relative survival for different followup periods demonstrates that patients with GCA experienced similar mortality to the general population (age-matched and sex-matched). Death from cardiovascular causes (45%) was the most common, followed by infection (17%) and cancer (17%). Infection was a significantly more common cause of death in the first year (chi-squared, p = 0.0002).Conclusion.Our population-based cohort study did not demonstrate any increased mortality risk for patients diagnosed with biopsy-proven GCA. The risk of death from infection early in the disease may be increased.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1069.1-1069
Author(s):  
L. Barra ◽  
J. Pope ◽  
P. Pequeno ◽  
J. Gatley ◽  
J. Widdifield

Background:Individuals with giant cell arteritis (GCA) are at increased risk of serious morbidity including cardiovascular disease and stroke. Yet the risk of mortality among individuals with GCA have produced conflicting reports1.Objectives:Our aim was to evaluate excess all-cause mortality among individuals with GCA relative to the general population over time.Methods:We performed a population-based study in Ontario, Canada, using health administrative data among all individuals 50 years and older. Individuals with GCA were identified using a validated case definition (81% PPV, 100% specificity). All Ontario residents aged 50 and above who do not have GCA served as the General Population comparators. Deaths occurring in each cohort each year were ascertained from vital statistics. Annual crude and age/sex standardized all-cause mortality rates were determined for individuals with and without GCA between 2000 and 2018. Standardized mortality ratios (SMRs) were calculated to measure relative excess mortality over time. Differences in mortality between sexes and ages were also evaluated.Results:Population denominators among individuals 50 years and older with GCA and the General Population increased over time with 12,792 GCA patients and 5,456,966 comparators by 2018. Annual standardized mortality rates among the comparators steadily declined over time and were significantly lower than GCA morality rates (Figure). Annual GCA mortality rates fluctuated between 42-61 deaths per 1000 population (with overlapping confidence intervals) during the same time period. SMRs for GCA ranged from 1.28 (95% CI 1.08,1.47) at the lowest in 2002 to 1.96 (95% CI 1.84, 2.07) at the highest in 2018. GCA mortality rates and SMRs were highest among males and younger age groups.Conclusion:Over a 19-year period, mortality has remained increased among GCA patients relative to the general population. GCA mortality rates were higher among males and more premature deaths were occurring at younger age groups. In our study, improvements to the relative excess mortality for GCA patients over time (mortality gap) did not occur. Understanding cause-specific mortality and other factors are necessary to inform contributors to premature mortality among GCA patients.References:[1]Hill CL, et al. Risk of mortality in patients with giant cell arteritis: a systematic review and meta-analysis. Semin Arthritis Rheum. 2017;46(4):513-9.Figure.Acknowledgments: :This study was supported by a CIORA grantDisclosure of Interests:Lillian Barra: None declared, Janet Pope Grant/research support from: AbbVie, Bristol-Myers Squibb, Eli Lilly & Company, Merck, Roche, Seattle Genetics, UCB, Consultant of: AbbVie, Actelion, Amgen, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Eicos Sciences, Eli Lilly & Company, Emerald, Gilead Sciences, Inc., Janssen, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, UCB, Speakers bureau: UCB, Priscila Pequeno: None declared, Jodi Gatley: None declared, Jessica Widdifield: None declared


2011 ◽  
Vol 29 (33) ◽  
pp. 4381-4386 ◽  
Author(s):  
Hui Miao ◽  
Helena M. Verkooijen ◽  
Kee-Seng Chia ◽  
Christine Bouchardy ◽  
Eero Pukkala ◽  
...  

Purpose Male breast cancer is a rare disease with an incidence rate less than 1% of that of female breast cancer. Given its low incidence, few studies have assessed risk and prognosis. Methods This population-based study, including 459,846 women and 2,665 men diagnosed with breast cancer in Denmark, Finland, Geneva, Norway, Singapore, and Sweden over the last 40 years, compares trends in incidence, relative survival, and relative excess mortality between the sexes. Results World standardized incidence rates of breast cancer were 66.7 per 105 person-years in women and 0.40 per 105 person-years in men. Women were diagnosed at a younger median age (61.7 years) than men (69.6 years). Male patients had a poorer 5-year relative survival ratio than women (0.72 [95% CI, 0.70 to 0.75] v 0.78 [95% CI, 0.78 to 0.78], respectively), corresponding to a relative excess risk (RER) of 1.27 (95% CI, 1.13 to 1.42). However, after adjustment for age and year of diagnosis, stage, and treatment, male patients had a significantly better relative survival from breast cancer than female patients (RER, 0.78; 95% CI, 0.62 to 0.97). Conclusion Male patients with breast cancer have later onset of disease and more advanced disease than female patients. Male patients with breast cancer have lower risk of death from breast cancer than comparable female patients.


2012 ◽  
Vol 72 (12) ◽  
pp. 1989-1994 ◽  
Author(s):  
Tanaz A Kermani ◽  
Kenneth J Warrington ◽  
Cynthia S Crowson ◽  
Steven R Ytterberg ◽  
Gene G Hunder ◽  
...  

ObjectivesTo evaluate incidence-trends and timing of large-vessel (LV) manifestations in patients with giant cell arteritis (GCA), and to examine the influence of LV manifestations on survival.MethodsA population-based incident cohort of patients diagnosed with GCA between 1950 and 2004 was used. LV involvement was defined as large-artery stenosis or aortic aneurysm/dissection that developed in the 1 year before GCA diagnosis or at any time thereafter. Patients were followed up until death or 31 December 2009.ResultsThe study included 204 patients, 80% women, mean age at diagnosis of GCA 76.0 years (±8.2 years). Median length of follow-up was 8.8 years. The cumulative incidence of any LV manifestation at 10 years was 24.9% for patients diagnosed with GCA between 1980 and 2004 compared with 8.3% for patients diagnosed with GCA between 1950 and 1979. The incidence of any LV event was high within the first year of GCA diagnosis. The incidence of aortic aneurysm/dissection increased 5 years after GCA diagnosis. Compared with the general population, survival was decreased in patients with an aortic aneurysm/dissection (standardized mortality ratio (SMR) 2.63; 95% CI 1.78 to 3.73) but not in patients with large-artery stenosis (SMR 1.44; 95% CI 0.87 to 2.25). Patients with GCA and aortic manifestations had a higher than expected number of deaths from cardiovascular and pulmonary causes than the general population. Among patients with GCA, aortic manifestations were associated with increased mortality (HR=3.4; 95% CI 2.2 to 5.4).ConclusionsVigilance and screening for aortic aneurysms should be considered in all patients 5 years after the incidence of GCA. Aortic aneurysm/dissection is associated with increased mortality in GCA.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 171.2-172
Author(s):  
R. Punekar ◽  
P. Lafontaine ◽  
J. H. Stone

Background:Giant cell arteritis (GCA) is a rare form of vasculitis usually manifesting in people aged ≥50 yr and is more common in women. Symptoms include headache, jaw claudication, fatigue, polymyalgia; and blindness if untreated. While risks of complications can be reduced with promptly administered high-dose glucocorticoids (GC; 20-60 mg for 2-4 wk, then slowly tapered), further risks of high GC exposure and related complications over the course of therapy remain.Objectives:To compare GC use and GC-related complications in GCA patients (pts) vs a general population (GnP) cohort.Methods:This retrospective, observational cohort study was based on Optum’s de-identified Clinformatics®Data Mart Database (01/01/06-30/06/18, study period). The GCA cohort included pts with ≥1 inpatient or ≥2 outpatient claims ≥30 days apart with GCA-related diagnosis codes (ICD-9: 446.5x/ICD-10: M31.6x) between 01/01/06-30/06/17 (pt identification period) during which first occurrence of a GCA-related medical claim was set as index date (ID). The GnP cohort included pts without any medical claims for rheumatoid arthritis, GCA or polymyalgia rheumatica diagnosis codes during the study period, with their ID set as 12 mo from start of continuous health plan enrollment. Pts in both cohorts were required to be age ≥50 yr (on the ID) with continuous health plan enrollment ≥12 mo pre- and post-ID. Cohorts were 1:1 propensity score matched. GC use and incidence of GC-related complications were assessed from GC initiation, starting from the baseline period (12-mo pre-ID) to the end of GC use during the post-index period (ie the end of data availability, end of the study period, or death, whichever occurred first). Descriptive analyses included mean, standard deviation (SD) and median values for continuous variables, and frequency (n and %) for categorical variables. Continuous variables were compared between matched cohorts usingt-tests and Wilcoxon sum rank tests. Categorical variables were compared between matched cohorts using Chi-square tests or Fisher’s exact tests. Duration of GC use was analyzed using the Kaplan-Meier method and compared between matched cohorts using log-rank tests.Results:There were 6071 pts included in each of the GCA and matched GnP cohorts; median age per cohort was 76 yr, median Elixhauser comorbidity index score was 3.0, and the majority (~75%) were women. The median follow-up duration was 44 and 48 mo in the GCA and GnP cohorts, respectively. A higher proportion of pts in the GCA cohort than the GnP cohort (90.6 vs 63.8%;p<0.001) used GC. The mean (SD) duration of GC therapy was 230.5 (±326.8) days in the GCA cohort vs 36.3 (±107.2) days in the GnP cohort (p<0.001). Although the mean (SD) daily dose of GC (prednisone equivalent) was similar in both cohorts (27.6 [±28.20] vs 27.7 [±25.18] mg), the mean (SD) cumulative GC dose was significantly higher in the GCA cohort than the GnP cohort (3503.0 (±4622.6) mg vs 503.7 (±1593.51) mg;p<0.001). This indicates that GCA pts had chronic GC exposure over the study period while GnP pts likely utilized higher dose GC burst therapy less frequently. The number of incident complications associated with GC use were significantly greater in the GCA cohort, and included hypertension, diabetes, skin toxicity, infections, neuropsychiatric effects, gastrointestinal complications, ocular effects, and cardiovascular disease (p<0.05).Conclusion:The overall GC burden in pts with GCA is significantly higher than the general population and may result in downstream complications related to GC exposure. The incidence of GC-related complications was statistically significantly higher in GCA pts compared with GnP pts, even with a short duration of GC use. The early onset of these complications may be a significant contributor to long-term healthcare costs in GCA pts.Acknowledgments:Study and medical writing (provided by Gauri Saal, MA, Economics, Prime, Knutsford, UK, under the direction of authors) were funded by Sanofi, Inc.Disclosure of Interests:Rajeshwari Punekar Shareholder of: Sanofi, Employee of: Sanofi, Patrick LaFontaine Shareholder of: Sanofi, Employee of: Sanofi, John H. Stone Grant/research support from: Roche, Consultant of: Roche


Rheumatology ◽  
2015 ◽  
Vol 55 (1) ◽  
pp. 33-40 ◽  
Author(s):  
Neda Amiri ◽  
Mary De Vera ◽  
Hyon K. Choi ◽  
Eric C. Sayre ◽  
J. Antonio Avina-Zubieta

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1567.1-1567
Author(s):  
T. Garvey ◽  
C. S. Crowson ◽  
M. Koster ◽  
E. Matteson ◽  
K. J. Warrington

Background:In previous studies patients with giant cell arteritis (GCA) have had survival rates that are similar to the general population.Objectives:To investigate survival trends and cause-specific mortality in patients diagnosed with GCA over a 60-year period.Methods:We assembled a population-based incidence cohort of patients with GCA diagnosed between 1950 and 2009. All patients were included if they met the American College of Rheumatology (ACR) 1990 Criteria for the Classification of GCA. Patients diagnosed between 2000 and 2009 could also be included if they met the following criteria: age greater than or equal to 50 years, elevated inflammatory markers, and radiographic evidence of large-vessel vasculitis attributed to GCA. A non-GCA comparison cohort was assembled from the same underlying population for each patient with GCA. Patients were followed until death, last contact, or December 31st, 2018. Survival trends were analyzed by grouping patients into the following categories according to year of GCA diagnosis: Group A 1950-1979; Group B 1980-1989; Group C 1990-1999; and Group D 2000-2009. Mortality rates were estimated using the Kaplan-Meier method and were compared with expected mortality rates for persons of the same age, sex, and calendar year, as estimated by regional population life tables. Cause-specific mortality was obtained from death certificates for patients in both cohorts. The causes were grouped according to ICD-9 chapters and hazard ratios were estimated against the non-GCA comparators.Results:The study population included 245 incident cases of GCA: 194 (79%) women and 51 (21%) men with mean age (±SD) of 76.2 (±8.3) years and median follow-up of 10.6 years. There was no overall difference in survival between the GCA cohort and the general population. The 2-, 5-, and 10-year survival rates (95% CI) were 89% (86, 93), 76% (70, 81), and 56% (50, 63) respectively with a standardized mortality ratio of 0.99 (0.86, 1.14). The standardized mortality ratios for Groups A, B, C, and D were 0.83 (0.57, 1.17), 0.92 (0.63, 1.3), 1.21 (0.85, 1.69), 0.70 (0.50, 0.95), respectively. The overall all-cause mortality adjusted for age, sex, and calendar-year was similar between the GCA patients and their comparators with a hazard ratio of 1.03 (0.84, 1.24). Mortality due to neoplasms was significantly lower in the GCA cohort with a hazard ratio of 0.53 (0.3, 0.92). Other cause-specific mortalities were not significantly different between the groups.Conclusion:In this population-based cohort of patients with GCA diagnosed over a 60-year period, the survival of patients diagnosed in recent years was significantly better than that of the general population. The explanation for this novel finding is unclear, but likely to be multifactorial. In this study the number of deaths due to neoplasm in the GCA group was significantly lower.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]Mohammad A. et al. Rate of comorbidities in Giant Cell Arteritis: A Population-based Study. J Rheumatol. 2017;44(1):84-90.[3]Salvarani C. et al. Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period. Arthritis Rheum. 2004;51(2):264-8.Acknowledgments: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 Grant/research support from: Pfizer research grant, Matthew Koster: None declared, Eric Matteson Grant/research support from: Pfizer, Consultant of: Boehringer Ingelheim, Gilead, TympoBio, Arena Pharmaceuticals, Speakers bureau: Simply Speaking, Kenneth J Warrington: None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 34.1-34
Author(s):  
R. S. Andev ◽  
N. Ahmad ◽  
A. Verdiyeva ◽  
R. Luqmani ◽  
S. Dubey

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


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 93.2-94
Author(s):  
L. C. Burg ◽  
P. Brossart ◽  
K. I. Reinking ◽  
R. P. Finger ◽  
C. Behning ◽  
...  

Background:Giant cell arteritis (GCA) is the most common form of systemic vasculitis in patients aged 50 years and older.1Visual symptoms such as amaurosis, diplopia, temporary or permanent visual field loss secondary to optic nerve ischemia are common manifestations.2The value of vascular ultrasound of extra-ocular vessels in diagnosing GCA is well established.3However, the role of transocular ultrasound of the central retinal artery in GCA patients has not yet been established.Objectives:To identify changes in flow velocities of the central retinal artery in GCA patients with visual symptoms and controls with transocular high resolution ultrasound.Methods:Prospective analysis of GCA patients with visual symptoms and controls. Ultrasound of the central retinal artery was performed in 18 newly diagnosed consecutive GCA patients with visual symptoms (GCA-group) and 25 controls without ocular pathology. Visual symptoms included amaurosis, diplopia and temporary or permanent visual field loss. For each eye, peak systolic values (PS) and end diastolic values (ED) were recorded. Furthermore, the resistance index of each central retinal artery was measured.Results:Twenty-one of 36 eyes of 18 GCA patients were affected. Therefore 21 central retinal were measured. The control group consisted of 50 central retinal arteries of 25 eye-healthy individuals. The mean age and gender distribution of the GCA-group were 75.6 years (SD± 8.1) with eight females (44 %) and 67 years (SD± 8.9) with twelve females (48%) in the control group. The mean flow velocity of the central retinal artery was PS 12.2 cm/s (SD± 3.5) and ED 3.7 cm/s (SD± 1.2) in the GCA group and PS 14.4 cm/s (SD± 3.2) and ED 5.1 cm/s (SD± 1.6) in the control group. The mean RI was 0.9 (SD± 0.3) in the GCA group and 0.8 (SD± 0.3) in the control group. Mean reduction in flow velocity in the GCA-group was PS 2.1 cm/s (p= 0.039) and ED 1.4 (p= 0.0004) cm/s, while the RI was increased by 0.14 (p= 0.077). The results for PS and ED measurements were statistically significant, while the results for RI were not significant.Conclusion:In GCA patients with ocular symptoms, a reduction of flow velocities of the central retinal artery compared to the eye-healthy control group was found. Results for PS and ED were significant. There seems to be a trend for decreased flow velocities in coexistence with visual symptoms in patients with GCA.References:[1]Warrington KJ, Matteson EL. Management guidelines and outcome measures in giant cell arteritis (GCA). Clin Exp Rheumatol 2007;25:137–41.[2]Chean CS, Prior JA, Helliwell T, et al. Characteristics of patients with giant cell arteritis who experience visual symptoms. Rheumatol Int 2019;39:1789–96.[3]Dejaco C, Ramiro S, Duftner C, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis 2018;77:636–43Figure 1.Transocular ultrasound of an affected eye in giant cell arteritis with reduced flow velocities and increased resistance index.Disclosure of Interests:None declared


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