Mortality rising for giant cell arteritis despite declines in general population
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The mortality rate of patients with giant cell arteritis increased from 50 deaths per 1,000 in 2000 to 57.6 per 1,000 in 2018, while mortality among the general population declined, according to data published in Arthritis Care & Research.
“Despite GCA and its treatment being associated with complications known to increase the risk of death, prior research on mortality among individuals with GCA has produced conflicting reports,” Lillian Barra, MD, FRCPC, MPH, of Western University, Schulich School of Medicine & Dentistry, in London, Ontario, and colleagues wrote. “A recent meta-analysis reported an increased risk of mortality in studies of GCA patients in hospitalized settings, but conflicting reports for population-based studies.”
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“The population studies in this meta-analysis included diverse methodologies, with studies often confined to inception cohorts of various follow-up, and variable use of general population comparators,” they added. “Moreover, some studies involving more contemporary cohorts have detected increased mortality risk in GCA, and conflicting reports on sex-related differences in mortality.”
To analyze all-cause mortality among patients with GCA compared with the general population over time, Barra and colleagues conducted a population-based study using health administrative data in Ontario, Canada. Using a validated case definition — with an 81% positive predictive value and 100% specificity — the researchers identified a cohort of 22,677 patients with GCA, aged 50 years or older, during the study period between April 1, 2000, and March 31, 2019. For the comparator group, the researchers used residents in the general population aged 50 years or older without GCA.
Barra and colleagues examined deaths using vital statistics, with annual crude, age/sex‐standardized and age‐ and sex‐specific all‐cause mortality rates determined for those with and without GCA between 2000 and 2018. The researchers also estimated standardized mortality ratios (SMRs).
According to the researchers, age‐ and sex‐standardized mortality rates were significantly higher for patients with GCA compared with the general population and demonstrated an increasing trend over time. Specifically, the mortality rate among patients with GCA increased from 50 (95% CI, 34-71.1) deaths per 1,000 in 2000 to 57.6 (95% CI, 50.8-65.2) deaths per 1,000 in 2018. Meanwhile, mortality rates among the general population significantly declined during this time.
Annual SMRs for patients with GCA generally increased over time, with the lowest ratio occurring in 2002 (1.22; 95% CI, 1.03-1.4) and the highest in 2018 (1.92; 95% CI, 1.81-2.03). Additionally, GCA mortality rates were more elevated for men compared with women.
“In this large population-based study, we found that GCA patients had a significantly higher annual risk of mortality compared to the general population over the 19-year study period,” Barra and colleagues wrote. “The increased mortality risk for GCA patients was seen in males and females and was most pronounced in individuals aged 50 to 65 years of age. Although mortality rates decreased over time for the general population, they trended upwards for GCA patents, resulting in higher SMRs for GCA patients in recent years.”
Perspective
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This study helps bring to light that the symptoms of GCA can be very severe and are not to be taken lightly. When a person complains of a new onset headache specific to the temporal area and/or jaw pain, it should be further evaluated to rule out GCA due to the possibility of more severe complications that can arise.
In practice, when seeing suspected GCA patients, it is important to keep in mind the mechanism of what is physiologically going on. GCA pain and discomfort arises from ischemia in relation to inflamed blood vessels, so decreasing the inflammation promptly is crucial, especially if the disease process may evolve to include eye involvement or aortic involvement that may not be known at the time of examination.
Not only can stroke and permanent blindness occur with the disease process, but also aortic aneurysms and dissections, which increase mortality rates. GCA is a diagnosis that when suspected or seen needs prompt attention and treatment.
The treatment for GCA is long-term corticosteroids, even if an arterial biopsy has been done but results have not returned. The downside of the treatment with long-term corticosteroids has a high risk of adverse events, such as infections, osteoporotic fractures, hypertension, diabetes, weight gain and other metabolic changes that can lead to cardiovascular events. IL-6 inhibitors have been also used to treat GCA in combination with corticosteroids.
Early recognition and prompt treatment with corticosteroids is key for early management and treatment of this disease process, which may also mortality rates.
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David A. McLain, MD, MACR, FACP, FRCP
This big data study relies on retrospective analysis of mortality of GCA ICD-coded patients vs. controls over 19 years. The mortality rate increased over this period for the GCA patients; however, this study was not able to determine the treatment that patients received.
Tocilizumab was not approved in the United States for GCA until 2017, and this study was conducted in Ontario, Canada from 2000-2018. Given this, it is likely that none of the patients received tocilizumab and that they only received corticosteroids with the associated toxicity. This study shows that developing a condition requiring high-dose corticosteroids at an advanced age is not favorable to one’s health.
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