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Objective: To estimate the additional healthcare system costs associated with giant cell arteritis (GCA) in the 1-year prediagnosis and postdiagnosis periods and over long-term follow-up compared to individuals with similar demographics and comorbidities without GCA., Methods: We performed a population-based study using health administrative data. Newly diagnosed cases of GCA (between 2002 and 2017 and aged ≥ 66 years) were identified using a validated algorithm and matched 1:6 to comparators using propensity scores. Follow-up data were accrued until death, outmigration, or March 31, 2020. The costs associated with care were determined across 3 phases: the year before the diagnosis of GCA, the year after, and ongoing costs thereafter in 2021 Canadian dollars (CAD)., Results: The cohort consisted of 6730 cases of GCA and 40,380 matched non-GCA comparators. The average age was 77 (IQR 72-82) years and 68.2% were female. A diagnosis of GCA was associated with an increased cost of CAD $6619.4 (95% CI 5964.9-7274.0) per patient during the 1-year prediagnostic period, $12,150.3 (95% CI 11,233.1-13,067.6) per patient in the 1-year postdiagnostic phase, and $20,886.2 (95% CI 17,195.2-24,577.2) per patient during ongoing care for year 3 onward. Increased costs were driven by inpatient hospitalizations, physician services, hospital outpatient clinic services, and emergency department visits., Conclusion: A diagnosis of GCA was associated with increased healthcare costs during all 3 phases of care. Given the substantial economic burden, strategies to reduce the healthcare utilization and costs associated with GCA are warranted., (Copyright © 2024 by the Journal of Rheumatology.)

Objective: Reports of mortality risks among individuals with giant cell arteritis (GCA) have been mixed. Our aim was to evaluate 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. We studied a cohort of 22,677 GCA patients ages ≥50 years that was identified using a validated case definition (with 81% positive predictive value, 100% specificity). General population comparators were residents ages ≥50 years without GCA. Deaths were ascertained from vital statistics. Annual crude, age- and sex-standardized, and age- and sex-specific all-cause mortality rates were determined for individuals with and without GCA between 2000 and 2018. Standardized mortality ratios (SMRs) were estimated., Results: Age- and sex-standardized mortality rates were significantly higher for GCA patients than comparators, and trending to increase over time with 50.0 deaths per 1,000 GCA patients in 2000 (95% confidence interval [95% CI] 34.0-71.1) and 57.6 deaths per 1,000 GCA patients in 2018 (95% CI 50.8-65.2), whereas mortality rates in the general population significantly declined over time. The annual SMRs for GCA patients generally increased over time, with the lowest SMR occurring in 2002 (1.22 [95% CI 1.03-1.40]) and the highest in 2018 (1.92 [95% CI 1.81-2.03]). GCA mortality rates were more elevated for male patients than female patients., Conclusion: Over a 19-year period, mortality rates were increased among GCA patients relative to the general population, and more premature deaths were occurring in younger age groups. The relative excess mortality for GCA patients did not improve over time., (© 2021 American College of Rheumatology.)

Objective: To estimate trends in the incidence and prevalence of GCA over time in Canada., Methods: We performed a population-based study of Ontario health administrative data using validated case definitions for GCA. Among Ontario residents ≥50 years of age we estimated the annual incidence and prevalence rates between 2000 and 2018. We performed sensitivity analyses using alternative validated case definitions to provide comparative estimates., Results: Between 2000 and 2018 there was a relatively stable incidence over time with 25 new cases per 100 000 people >50 years of age. Age-standardized incidence rates were significantly higher among females than males [31 cases (95% CI: 29, 34) vs 15 cases (95% CI: 13, 18) per 100 000 in 2000]. Trends in age-standardized incidence rates were stable among females but increased among males over time. Incidence rates were highest among those ≥70 years of age. Standardized prevalence rates increased from 125 (95% CI 121, 129) to 235 (95% CI 231, 239) cases per 100 000 from 2000 to 2018. The age-standardized rates among males rose from 76 (95% CI 72, 81) cases in 2000 to 156 (95% CI 151, 161) cases per 100 000 population in 2018. Between 2000 and 2018, the age-standardized rates among females similarly increased over time, from 167 (95% CI 161, 173) to 304 (95% CI 297, 310) cases per 100 000 population., Conclusion: The incidence and prevalence of GCA in Ontario is similar to that reported in the USA and northern Europe and considerably higher than that reported for southern Europe and non-European populations., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Takayasu arteritis (TA) is a rare large-vessel vasculitis that puts patients at high risk of developing severe ischemic events (SIE). Outcomes for TA patients with SIE are poorly understood. We aim to describe the characteristics of TA patients experiencing SIE., Methods: All TA patients with at least 1 followup visit seen between 1988 and 2015 were included from 3 academic centers in Ontario, Canada. Diagnosis was based on American College of Rheumatology criteria, physician opinion, and vascular imaging. SIE were defined as cerebrovascular accident (CVA), acute coronary syndrome (ACS), ischemic cardiomyopathy, ischemic blindness, and/or ischemic bowel or limb requiring surgery., Results: Of the 52 patients with TA included in the study, 51 (98%) were female and 22 (42%) were of European descent. The mean age was 31 (SD 12) at the time of diagnosis and the followup time was 6 years (SD 5). Fifteen (29%) experienced an SIE: 5 CVA, 5 ACS, 1 ischemic cardiomyopathy, and 4 limb ischemia. Thirteen out of 15 SIE (87%) occurred at or before diagnosis. Patients with SIE were more likely than those without SIE to be started on corticosteroids combined with immunosuppressants (p = 0.04) and antiplatelet agents (p = 0.004). Outcomes including disease activity and damage scores were similar between patients with and without SIE., Conclusion: SIE are common in patients with TA and occur early in the disease. With aggressive treatment, patients with SIE had a favorable prognosis.

Objective: To compare the clinical features, efficacy and safety of treatment regimens, and outcomes of childhood- and adult-onset Takayasu arteritis (TAK)., Methods: The study was designed as a retrospective cohort study comparing patients with childhood-onset TAK (from 1986 onward) to patients with adult-onset TAK (from 1988 onward) who were followed up until 2014 or 2015 at 4 centers in Ontario, Canada. Demographic, clinical, laboratory, and angiographic features, treatment regimens, and outcomes were recorded throughout the course of the disease. Disease activity and damage scores were completed retrospectively., Results: Twenty-nine children and 48 adults (median age at diagnosis 12.1 years and 31.2 years, respectively) were included. A lower predominance of females was observed among the childhood-onset TAK cohort (76% versus 100% of patients with adult-onset TAK; P < 0.01), and children had a shorter delay to diagnosis (median 6.0 months versus 12.2 months for adults; P = 0.03). The distribution of vascular involvement was also different, with children having significantly more aortic and renal artery involvement and a higher frequency of arterial hypertension. Relapses in the first year after diagnosis were common both in children (39%) and in adults (28%). Two children, but no adults, died., Conclusion: Childhood-onset TAK has a lower female predominance and a higher frequency of aortic and renal involvement compared to adult-onset TAK. Relapses and disease burden were high in both groups, corroborating the need for careful monitoring of disease activity and aggressive therapeutic management., (© 2018, American College of Rheumatology.)