Risk for COVID-19-Related Death Elevated in Rare Autoimmune Rheumatic Diseases

Compared with the general population, patients with rare autoimmune rheumatic diseases have more than a 2-fold increased risk for COVID-19-related death.

Patients with rare autoimmune rheumatic diseases (AIRDs) vs the general population have a significantly higher risk for COVID-19-related death, with the risk further increasing with corticosteroid use, according to study results published in Rheumatology.

Researchers collected data from Hospital Episode Statistics, a nationwide database with information on every episode of admitted care at public hospitals in England.

Rare AIRDs of interest included antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, Behçet disease, giant cell arteritis, idiopathic inflammatory myopathies, juvenile inflammatory arthritis, scleroderma, systemic lupus erythematosus, and Takayasu arteritis.

The outcome of interest was death from COVID-19 infection, which was determined using linked national health records. The UK government’s online COVID-19 dashboard was used to calculate infection and death rates in the general population.

The association between 30-day corticosteroid use and COVID-19-related death was also assessed; risk ratios were stratified by prednisolone equivalent dose.

The study cohort included 168,330 patients with rare AIRD diagnoses, among whom 118,199 (70.2%) were women. Median age among the cohort was 61.7 years.

This has important implications for people living with RAIRD, their clinicians and for public health policy; to protect their health and reduce the risk of severe outcomes.

Between August 2020 and April 2021, a total of 9961 (5.92%) of the rare AIRD cohort had a positive molecular test for COVID-19, with the infection rate in the general population at 6.13%.

During the study period, 5822 patients (3.5%) with a rare AIRD died, among whom 1342 (0.80%) had COVID-19 listed on their death certificate. However, the rate of COVID-19-associated death was much smaller in the general population at 0.15%.

The use of corticosteroids was found to increase the risk for COVID-19-associated death in patients with rare AIRDs. The relationship was dose-dependent, with a 1.10-fold (95% CI, 1.08-1.13) increase in risk for death for each added 5 mg prednisolone equivalent dose. Patients receiving more than 15 mg prednisolone equivalent dose daily at the time of COVID-19 infection were 2.15 (95% CI, 1.80-2.56) times more likely to die than patients not receiving corticosteroids (P <.01).

Results from the study highlighted the impact of COVID-19 on patients with certain AIRDs. The risk for COVID-19-related death was significantly elevated in patients with rare AIRDs compared with the general population. Corticosteroid use appeared to further increase the risk for death.

One of the study limitations was that information on other immunosuppressant medications was not collected.

“This has important implications for people living with [rare] AIRD, their clinicians and for public health policy; to protect their health and reduce the risk of severe outcomes,” the study authors wrote.

“[Rare] AIRD may require immunosuppression with corticosteroids. We have demonstrated that corticosteroids are associated with poorer COVID-19 outcomes, and this should form part of the decision-making process when considering a corticosteroid prescription,” they concluded.

Disclosure: Multiple study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures. 


Rutter M, Lanyon PC, Grainge MJ, et al. COVID-19 infection, admission and death and the impact of corticosteroids amongst people with rare autoimmune rheumatic disease during the second wave of covid-19 in England: results from the RECORDER Project. Rheumatology (Oxford). Published online April 5, 2023. doi:10.1093/rheumatology/kead150