AIM 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. METHODS 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. RESULTS 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 < .001 for all) and increased for SS (+1.53%/year, p = .01). The worldwide ASMR of SLE decreased significantly between 2001 and 2003 (-6.37%, p < .05) before increasing slightly between 2004 and 2014 (+0.58%, p < .01). CONCLUSIONS We observed a strong heterogeneity of standardized mortality rates across all countries analyzed for 6 autoimmune diseases. Those results further highlight the impact of world-wide inequities and major gaps in access to care and strategies for diagnosis and management of rare diseases, a crucial finding for world-wide physicians, patient associations and policy makers.

中文翻译：

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2001年至2014年间全球自身免疫性系统疾病全因死亡率的趋势。

目的描述6种自身免疫性系统疾病（AISD）在2001-2014年死亡率的变化，即系统性红斑狼疮（SLE），系统性硬化症（SSc），特发性炎症性肌病（IIM），干燥综合征（SS），混合性结缔组织病（MCTD）和与ANCA相关的血管炎（AAV）在国家，大陆和世界范围内。方法基于ICD-10代码，从世界卫生组织（WHO）死亡率数据库中检索出每种疾病的死亡率数据。我们将年龄标准化死亡率（ASMR）计算为每百万居民的估计死亡人数及其95％的置信区间（95％CI）。使用多元Poisson回归分析了性别，地理区域和特定疾病死亡率之间的关联。使用Jointpoint软件分析了2001-2014年的时间趋势。结果2014年，全球SLE的ASMR死亡/百万居民为2.68（95％CI：2.62-2.75），SSc为1.46（1.42-1.51），IIM为0.47（0.44-0.49），0.17（0.15-0.18） SS，MCTD为0.11（0.10-0.13），AAV为0.53（0.50-0.56），其中欧洲的ASMR通常低于北美，拉丁美洲和亚洲。在2001年至2014年之间，SSc（-0.71％/年），IIM（-1.65％/年）和AAV（-1.01％/年；所有p <0.001）的全球ASMR显着下降，而SS（+ 1.53％/年，p = 0.01）。SLE的全球ASMR在2001年至2003年期间显着下降（-6.37％，p <.05），然后在2004年至2014年之间略有上升（+ 0.58％，p <.01）。结论我们观察到在分析了6种自身免疫性疾病的所有国家中，标准化死亡率存在很大的异质性。