Giant-cell arteritis-related mortality in France: A multiple-cause-of-death analysis
Introduction
Giant—cell arteritis (GCA) is a large—vessel vasculitis usually affecting individuals over 50 years old, typically presenting with unilateral or bilateral headache, myalgias, fatigue, fever, weight loss, and sometimes acute vision loss [1]. It is frequently associated with polymyalgia rheumatica (PMR), which represents either a different manifestation of the same disease or overlapping conditions. Both diseases are associated with an inflammatory state with an increased erythrocyte sedimentation rate (ESR) and/or C—reactive protein (CRP), and the treatment relies mainly on corticosteroids, usually prescribed for a prolonged period. GCA seems to affect predominantly European populations, especially those of northern European descent, and is slightly more common in women than in men, with a lifetime risk of GCA estimated at 1.0% in women versus 0.5% in men [2,3]. GCA is the most frequent primary vasculitis with an estimated incidence in the United States of 18 per 100,000, but data based on recent studies suggest that GCA incidence has stabilized or even decreased in recent years [4,5]. Data regarding GCA mortality are controversial. A recent meta—analysis including 17 studies found no difference in long—term mortality between GCA patients and the general population (mortality odds ratio of 1.03), except in GCA patients recruited through a hospital setting (mortality odds ratio of 1.61), especially in the first two years following GCA diagnosis [6]. Mortality in GCA has been attributed to specific complications of the disease, particularly aortic aneurysms and cerebrovascular events associated with a lower survival [5,7,8]. Some studies also showed increased cardiovascular mortality and increased mortality due to infectious diseases in GCA patients compared with general population [9,10].
To better characterize mortality causes in GCA, we examined data from death certificates compiled by the French Epidemiological Centre on Medical Causes of Death (CépiDc, Institut National de la Santé et de la Recherche Médicale) between 2005 and 2014. Using multiple— cause—of—death (MCOD) analysis, we calculated age—adjusted mortality rates for GCA, examined differences in mortality rates according to age and gender and recorded the underlying causes of death (UCD).
Section snippets
Dataxtraction
Data were extracted from the Institut National de la Statistique et des études économiques (INSEE) database for population analysis and from the CépiDc database for causes of death analysis. Since 2000, CépiDc has recorded all death certificates issued in France, and these anonymized data are available to researchers. French death certificates consist of 2 parts and comply with the World Health Organization standards. Part I lists the “diseases related to the morbid process leading to death” in
Numbers of deaths with GCA in France between 20015 and 2014
Between 2005 and 2014, 5,311,098 adults died in France. Among them, the CépiDc recorded 4,628 death certificates reporting a diagnosis of GCA as UCD or NUCD. The absolute numbers of deaths with GCA listed as UCD or NUCD, stratified by year during the study period, are shown in Table 1. The mean number of annual deaths was 462 (±33.4) and 51.2% of them occurred in hospital. The mean age of death was 86 (±6.8) years: 84.6 (±6.9) years in men and 86.7 (±6.6) years in women (p < 0.001). Throughout
Discussion
Using the CépiDc database containing all French death certificates, we found that there was no significant difference in the age repartition of death when GCA was mentioned in the death certificate compared to that of the general population, suggesting that GCA is associated with a survival reflecting the age incidence of this disease. These results are consistent with a recent international meta—analysis that found that long—term mortality was not increased in GCA [3,6]. In a Northern Italian
Conclusions
Despite limitations, our study suggests that overall mortality is not strongly increased in GCA patients and remained stable between 2005 and 2014, suggesting that there was no improvement in the treatments during the period. Most common comorbidities were cardiovascular and infectious diseases, which can both be considered complications of GCA but also long—term complications of GCA treatment, especially corticosteroid treatment. Improving our knowledge on mortality in GCA will help determine
Authors contributions
F.C.A., and Z.A. designed the study.
T.C., R.L., G.R., M.E. and F.C.A. collected the data.
R.L. and F.C.A. conducted the statistical analysis.
T.C., R.L., G.R., J.H., A.M., M.E., Z.A. and F.C.A. analyzed and interpreted the data. T.C., R.L., G.R. and F.C.A. wrote the manuscript.
All authors critically reviewed and approved the final version of the manuscript.
Acknowledgement
This work did not receive any financial support.
Conflicts of interest
The authors declare they have no conflicts of interest to report.
References (18)
- et al.
Epidemiology and natural.history of giant cell arteritis
Rev Med Interne
(2017) - et al.
Risk of mortality in patients with giant cell arteritis: A systematic review and meta-analysis
Semin Arthritis Rheum
(2017) - et al.
Cerebrovascular accident in patients with giant cell arteritis: A systematic review and meta analysis of cohort studies
Semin Arthritis Rheum
(2016) - et al.
Venous thrombosis in patients with giant cell arteritis: Features and outcomes in a cohort study
Joint Bone Spine
(2017) - et al.
Clinical practice. Giant-cell arteritis and polymyalgia rheumatica
N Engl J Med
(2014) - et al.
The lifetime risk of adult- onset rheumatoid arthritis and other inflammatory autoimmune rheumatic diseases
Arthritis Rheum
(2011) - et al.
Incidence and mortality rates of biopsy-proven giant cell arteritis in southern Sweden
Ann Rheum Dis
(2015) - et al.
Large-vessel involvement in giant cell arteritis: A population-based cohort study of the incidence-trends and prognosis
Ann Rheum Dis
(2013) - et al.
Cerebrovascular Events are Associated With Lower Survival in Giant Cell Arteritis: A Case-Controlled Multicenter Study, Joint, Bone, Spine : Revue du rhumatisme
(2017)
Cited by (16)
Efficacy and safety of steroid-sparing treatments in giant cell arteritis according to the glucocorticoids tapering regimen: A systematic review and meta-analysis
2021, European Journal of Internal MedicineCitation Excerpt :This situation contributed to the lack of power and decreased the quality of evidence. Because of the associated iatrogenic effects, it is crucial to demonstrate the validity of steroid-sparing treatments [1], particularly in patients with GCA who are often elderly and comorbid [8]. Tocilizumab seems to be effective for GC sparing in GCA.
An overview of the perspectives on experimental models and new therapeutic targets in giant cell arteritis
2020, Autoimmunity ReviewsCitation Excerpt :GCA is not responsible for increased mortality compared to the general population [31–33,35,36], although patients with GCA presenting with vision loss, aortic aneurysms and/or dissection appear to have a worse prognosis [36–39]. The main causes of death of patients with GCA are cardiovascular diseases, followed by cerebrovascular disease, infections and malignancy [35,38,40]. Of note, some studies described the association of GCA with another systemic disease, such as myelodysplastic syndrome or inflammatory bowel diseases [41,42].
Serum amyloid A as a marker of disease activity in Giant cell arteritis
2020, Autoimmunity ReviewsSurvival of patient with giant cell arteritis: Impact of vision loss and treatment with aspirin
2019, Autoimmunity ReviewsNeurologic manifestations of giant cell arteritis
2022, Journal of Neurology
- 1
These authors contributed equally to this work and are co--first authors.