All-cause and cause-specific mortality in people with rare autoimmune rheumatic diseases in England using whole population data
Authors' conclusion
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.
Study context
- Population
- All individuals in England with a rare autoimmune rheumatic diseases (RAIRD) diagnosis recorded in hospital admissions data and alive in 201353,237 deaths among people with RAIRD, cohort 69.9% female
- Exposure
- Rates were compared with Office for National Statistics data for the general population of England
- Outcome
- Deaths among people with RAIRD
- Follow-up
- 2013 to 2020
Methodological detailsusing linked routinely collected healthcare data in England, crude and age-sex-standardised mortality rates
- Using linked routinely collected healthcare data in England
- Crude and age-sex-standardised mortality rates
Main finding
As reported in the abstract
RAIRD
53,237
events
Observational data: associations, not effects.
Further findings
| Outcome | Estimate | |
|---|---|---|
| All-cause mortality, age-sex-standardised mortality rate ratio · 2013 | age-sex-standardised mortality rate ratio 2.6 (95% CI 2.6–2.7) | |
| All-cause mortality, age-sex-standardised mortality rate ratio · 2020 | age-sex-standardised mortality rate ratio 2.3 (CI 2.2–2.3) | |
| Mortality from infection | rate ratio 4.10 (CI 3.81–4.39) | |
| Mortality from cardiovascular disease | rate ratio 2.50 (CI 2.45–2.55) | |
| Mortality from COVID-19 | rate ratio 2.74 (CI 2.59–2.89) | |
| Mortality from respiratory disease | rate ratio 2.90 (CI 2.82–2.98) | |
| 0.20.5125Ratio (log scale) |
All-cause mortality trend · 2013 to 2020
- All-cause mortality declined between 2013 and 2019 but rose in 2020 during the COVID-19 pandemic
Cohort characteristics
- Female
- 69.9%
- Median age rose from 61.5 to 62.5 years during the study
All-cause mortality by disease
- Highest all-cause mortality occurred in systemic sclerosis, polymyositis/dermatomyositis, microscopic polyangiitis and unspecified arteritis
Deaths from opportunistic infections
- Deaths from opportunistic infections were rare
Original publication
All-cause and cause-specific mortality in people with rare autoimmune rheumatic diseases in England using whole population data. Rheumatology (Oxford). Published 3 November 2026. doi:10.
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