All-cause and cause-specific mortality in people with rare autoimmune rheumatic diseases in England using whole population data
Megan Rutter, Peter Stilwell, Charlotte Eversfield, Peter C Lanyon, Matthew J Grainge, Richard Hubbard, Mary Bythell, Jeanette Aston, Sean McPhail, Sarah Stevens, Fiona A PearceAbstract
Objectives
People with rare autoimmune rheumatic diseases (RAIRD) experience morbidity and premature mortality due to disease activity and immunosuppression. We conducted a nationwide, whole-population retrospective cohort study using linked routinely collected healthcare data in England.
Methods
All individuals in England with a RAIRD diagnosis recorded in hospital admissions data and alive in 2013 were included. Linked demographic and death certificate data were used to calculate crude and age–sex–standardised mortality rates for all causes and leading causes of death from 2013 to 2020. Rates were compared with Office for National Statistics data for the general population of England.
Results
There were 53,237 deaths among people with RAIRD. The cohort was 69.9% female, median age rose from 61.5 to 62.5 years during the study. All-cause mortality declined between 2013 and 2019 but rose in 2020 during the COVID-19 pandemic. The age–sex–standardised mortality rate ratio versus the general population decreased from 2.6 (95% CI 2.6–2.7) in 2013 to 2.3 (2.2–2.3) in 2020. Mortality was particularly elevated for infection (rate ratio 4.10, 3.81–4.39), cardiovascular disease (2.50, 2.45–2.55), COVID-19 (2.74, 2.59–2.89) and respiratory disease (2.90, 2.82–2.98). Highest all-cause mortality occurred in systemic sclerosis, polymyositis/dermatomyositis, microscopic polyangiitis and unspecified arteritis. Deaths from opportunistic infections were rare.
Conclusions
Mortality in RAIRD remains more than twice that of the general population despite modest improvement over time. Infection, respiratory disease and COVID-19 remain major contributors, highlighting the need for targeted infection prevention and cardiovascular risk management.