Background/Purpose: Giant Cell Arteritis (GCA) is the most common primary vasculitis in high income countries and ischemic complications of GCA include blindness and stroke. The impact of GCA on mortality is unclear, with conflicting evidence as to whether mortality is increased. We utilized whole-population linked health data to investigate all-cause mortality and the contributing causes of death of hospitalised GCA patients compared to matched hospital controls.

Methods: All hospital presentations with GCA from 1980 to 2015 were identified. Each patient (n=1,802) was propensity score matched with hospital-based controls (n=3,945). Data from the from the Death Registrations database were used to determine all-cause and contributing causes of mortality. The association between GCA and mortality was assessed using Cox regression models. Standardized mortality ratios (SMR) were produced for the GCA cohort compared to the general population of Western Australia.

Results: GCA patients had increased unadjusted (HR 1.58, 95% CI 1.46, 1.71; P< 0.001), and multivariable-adjusted risk of mortality (aHR 1.13, 95%CI 1.04, 1.23; P< 0.001) compared to controls. GCA patients requiring hospital care had an increased likelihood of mortality compared to the general population for up to ten years, most pronounced in the first year (SMR 2.67, 95%CI 2.30, 3.10; p< 0.001). Adjusting for age, sex and IA status, people with GCA had an increased likelihood of 18-year deaths related to diseases of the musculoskeletal, connective tissue and subcutaneous system diseases (excluding GCA); diseases of the eye and adnexa; diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism; diseases of the genitourinary system; certain infectious and parasitic diseases; diseases of the digestive system; endocrine, nutritional and metabolic diseases; diseases of the respiratory system; diseases of the circulatory system. Adjusting for age, sex and IA status, people with GCA had a decreased likelihood of 18-year deaths related to neoplasm and diseases of the central nervous system.

Conclusion: Western Australians presenting to hospital with GCA from 1980 to 2015 had increased mortality compared to matched controls. The increased risk of death is likely related to both the disease, and complications of steroid therapies; both more efficacious and less harmful therapies are required to reduce mortality in GCA.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/all-cause-mortality-in-a-hospital-ascertained-cohort-with-giant-cell-arteritis-a-longitudinal-population-level-data-linkage-cohort-study/