Worldwide trends in all-cause mortality of auto-immune systemic diseases between 2001 and 2014

To describe changes in the 2001–2014 mortality of 6 autoimmune systemic diseases (AISDs), namely Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc), Idiopathic Inflammatory Myopathies (IIM), Sjögren's Syndrome (SS), Mixed Connective Tissue Disease (MCTD) and ANCA-associated vasculitis (AAV) at the country-, continent-, and world-levels. Mortality data were retrieved from the World Health Organization (WHO) mortality database for each disease, based on ICD-10 codes. We computed age-standardized mortality rate (ASMR) as the estimated number of deaths per million inhabitants and its 95% confidence interval (95%CI). The association between gender, geographical areas and disease-specific mortality was analyzed using multivariate Poisson regression. The 2001–2014 temporal trends were analyzed using Jointpoint software. In 2014, the worldwide ASMR for SLE was 2.68 (95%CI: 2.62–2.75) deaths/millions inhabitants, 1.46 (1.42–1.51) for SSc, 0.47 (0.44–0.49) for IIM, 0.17 (0.15–0.18) for SS, 0.11 (0.10–0.13) for MCTD and 0.53 (0.50–0.56) for AAV, with ASMRs generally lower in Europe than in North America, Latin America and Asia. Between 2001 and 2014, the worldwide ASMR decreased significantly for SSc (−0.71%/year), IIM (−1.65%/year) and AAV (−1.01%/year; p