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...

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Veröffentlicht in:Autoimmunity reviews 2020-06, Vol.19 (6), p.102531, Article 102531
Hauptverfasser: Scherlinger, Marc, Mertz, Philippe, Sagez, Flora, Meyer, Alain, Felten, Renaud, Chatelus, Emmanuel, Javier, Rose-Marie, Sordet, Christelle, Martin, Thierry, Korganow, Anne-Sophie, Guffroy, Aurélien, Poindron, Vincent, Richez, Christophe, Truchetet, Marie-Elise, Blanco, Patrick, Schaeverbeke, Thierry, Sibilia, Jean, Devillers, Hervé, Arnaud, Laurent
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container_issue 6
container_start_page 102531
container_title Autoimmunity reviews
container_volume 19
creator Scherlinger, Marc
Mertz, Philippe
Sagez, Flora
Meyer, Alain
Felten, Renaud
Chatelus, Emmanuel
Javier, Rose-Marie
Sordet, Christelle
Martin, Thierry
Korganow, Anne-Sophie
Guffroy, Aurélien
Poindron, Vincent
Richez, Christophe
Truchetet, Marie-Elise
Blanco, Patrick
Schaeverbeke, Thierry
Sibilia, Jean
Devillers, Hervé
Arnaud, Laurent
description 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 
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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 &lt; .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 &lt; .05) before increasing slightly between 2004 and 2014 (+0.58%, p &lt; .01). 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. •The age-standardized mortality rate (ASMR) of 6 auto-immune diseases was computed using the WHO mortality database.•The ASMR were generally lower in Europe than in North America, Latin America and Asia.•Between 2001 and 2014, there was a significant worldwide decrease of the ASMR of SSc, IIM &amp; AAVs while it increased for SLE and Sjögren's</description><identifier>ISSN: 1568-9972</identifier><identifier>EISSN: 1568-9972</identifier><identifier>EISSN: 1873-0183</identifier><identifier>DOI: 10.1016/j.autrev.2020.102531</identifier><identifier>PMID: 32234406</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Autoimmune diseases ; Epidemiology ; Immunology ; Life Sciences ; Mortality ; Trends</subject><ispartof>Autoimmunity reviews, 2020-06, Vol.19 (6), p.102531, Article 102531</ispartof><rights>2020</rights><rights>Copyright © 2020. 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The worldwide ASMR of SLE decreased significantly between 2001 and 2003 (−6.37%, p &lt; .05) before increasing slightly between 2004 and 2014 (+0.58%, p &lt; .01). We observed a strong heterogeneity of standardized mortality rates across all countries analyzed for 6 autoimmune diseases. 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1568-9972
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subjects Autoimmune diseases
Epidemiology
Immunology
Life Sciences
Mortality
Trends
title Worldwide trends in all-cause mortality of auto-immune systemic diseases between 2001 and 2014
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