Elsevier

Autoimmunity Reviews

Volume 17, Issue 12, December 2018, Pages 1219-1224
Autoimmunity Reviews

Giant-cell arteritis-related mortality in France: A multiple-cause-of-death analysis

https://doi.org/10.1016/j.autrev.2018.06.012Get rights and content

Abstract

Objectives

Giant—cell arteritis (GCA) is a large vessel vasculitis. Data regarding mortality are controversial. We describe the mortality data of the French death certificates for the period of 2005 to 2014.

Methods

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 analyzed the underlying causes of death (UCD).

Results

We analyzed 4628 death certificates listing a diagnosis of GCA as UCD or non-underlying cause of death (NUCD). The mean age of death was 86 (±6.8) years. The overall age—standardized mortality rate among GCA patients was 7.2 per million population. Throughout the study period, the mean age of death was significantly increased (r = 0.17, p < .0001) in both genders. There was no significant difference with age repartition of death in the general population (p = .26). When GCA was listed as the UCD, most frequent associated diseases were cardiovascular (79%) and infectious diseases (35%). When GCA was reported as the NUCD, the listed UCD was a cardiovascular event in 40% of cases, neoplasm in 13%, neurodegenerative disorder in 11% and infectious disease in 10%. When GCA was the UCD or NUCD, an age—adjusted observed/expected ratio > 1 in GCA—associated mortality compared with the general population mortality was observed for tuberculosis, pneumonia and cardiovascular diseases.

Conclusion

In this analysis of French death certificates mentioning GCA, we observed a stable standardized mortality rate between 2005 and 2014. The most frequent associated diseases were cardiovascular diseases and infections.

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)

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These authors contributed equally to this work and are co-­-first authors.

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