Death certificates underestimate the true burden of rare diseases: the example of lupus

What is it about?

Systemic lupus erythematosus (“lupus”) is a rare disease of the immune system that affects mostly women in childbearing age. The risk of premature death is greatly increased in individuals with lupus compared to the general population. Death certificate data are frequently used to measure disease burden for public health planning and allocation of health resources. We hypothesized that for rare diseases (lupus as an example) death certificate data might not be a good resource to estimate the true burden of disease. Rare diseases present in a variety of forms and are associated with a large number of comorbidities that may mask the underlying disease. To study this, we identified the death certificates of decedents from a large and unique cohort of individuals with lupus and their general population comparators in Sweden. We calculated that 59% of 1802 decedents with an established diagnosis of lupus during life had lupus not stated on their death certificate. Older age at death and cancer on the death certificate were associated with not coding lupus as a cause of death in lupus decedents. On the other hand, renal failure was more likely to appear together with lupus on the death certificate.

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Why is it important?

With the example of systemic lupus erythematosus, we have shown that it is inadequate to solely rely on death certificate data to study the burden of disease. Same applies to the use of those data for research purposes. Plans for improving the reporting of rare diseases on death certificates should be sought by public health authorities to ensure better utility of those data.

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