Systemic autoimmune disease as a cause of death: mortality burden and comorbidities

Rheumatology (Oxford). 2021 Mar 2;60(3):1321-1330. doi: 10.1093/rheumatology/keaa537.

Abstract

Objectives: Systemic autoimmune diseases (SAIDs) have chronic trajectories and share characteristics of self-directed inflammation, as well as aspects of clinical expression. Nonetheless, burden-of-disease studies rarely investigate them as a distinct category. This study aims to assess the mortality rate of SAIDs as a group and to evaluate co-occurring causes of death.

Methods: We used death certificate data in the Netherlands, 2013-2017 (N = 711 247), and constructed a SAIDs list at the fourth-position ICD-10 level. The mortality rate of SAIDs as underlying cause of death (CoD), non-underlying CoD, and any-mention CoD was calculated. We estimated age-sex-standardized observed/expected (O/E) ratios to assess comorbidities in deaths with SAID relative to the general deceased population.

Results: We observed 3335 deaths with SAID on their death certificate (0.47% of all deaths). The mortality rate of SAID was 14.6 per million population as underlying CoD, 28.0 as non-underlying CoD, and 39.7 as any-mention CoD. The mortality rate was higher for females and increased exponentially with age. SAID-related deaths were positively associated with all comorbidities except for solid neoplasms and mental conditions. Particularly strong was the association with diseases of the musculoskeletal system (O/E = 3.38; 95% CI: 2.98, 3.82), other diseases of the genitourinary system (O/E = 2.73; 95% CI: 2.18, 3.38), influenza (O/E = 2.71; 95% CI: 1.74, 4.03), blood diseases (O/E = 2.02; 95% CI: 1.70, 2.39), skin and subcutaneous tissue diseases (O/E = 1.95; 95% CI: 1.54, 2.45), and infectious diseases (O/E = 1.85; 95% CI: 1.70, 2.01).

Conclusion: Systemic autoimmune diseases constitute a rare group of causes of death, but contribute to mortality through multiple comorbidities. Classification systems could be adapted to better encompass these diseases as a category.

Keywords: autoimmune diseases; comorbidity; epidemiology; mortality; population studies.

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / mortality*
  • Cause of Death
  • Comorbidity
  • Cost of Illness
  • Female
  • Humans
  • Male
  • Middle Aged
  • Netherlands / epidemiology
  • Risk Factors
  • Sex Factors