Marc Scherlinger1, Philippe Mertz2, Flora Sagez2, Alain Meyer2, Renaud Felten2, Emmanuel Chatelus2, Rose-Marie Javier2, Christelle Sordet2, Thierry Martin3, Anne-Sophie Korganow3, Aurélien Guffroy3, Vincent Poindron4, Christophe Richez1, Marie-Elise Truchetet1, Patrick Blanco5, Thierry Schaeverbeke6, Jean Sibilia5, Hervé Devillers7, Laurent Arnaud8. 1. Centre Hospitalier Universitaire de Bordeaux, FHU ACRONIM, Place Amélie Raba Léon, 33076 Bordeaux, France; CNRS-UMR 5164 ImmunoConcEpT, 146 rue Léo Saignat, 33076 Bordeaux, France; Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France. 2. Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France; Service de rhumatologie, Centre Hospitalier Universitaire de Strasbourg, 1 avenue Molière, 67098 Strasbourg, France. 3. Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France; Service d'immunologie clinique, Centre Hospitalier Universitaire de Strasbourg, Strasbourg, France; Immuno-rhumatologie moléculaire, INSERM UMR-S 1109, Strasbourg, France. 4. Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France; Service d'immunologie clinique, Centre Hospitalier Universitaire de Strasbourg, Strasbourg, France. 5. CNRS-UMR 5164 ImmunoConcEpT, 146 rue Léo Saignat, 33076 Bordeaux, France; Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France. 6. Centre Hospitalier Universitaire de Bordeaux, FHU ACRONIM, Place Amélie Raba Léon, 33076 Bordeaux, France; CNRS-UMR 5164 ImmunoConcEpT, 146 rue Léo Saignat, 33076 Bordeaux, France. 7. Service De Médecine Interne et Maladies Systémiques (médecine interne 2), Centre Hospitalier Universitaire de Dijon, Hôpital François-Mitterrand, Dijon, France; Centre d'investigation clinique - Epidémiologie Clinique, INSERM CIC 1432, Hôpital François-Mitterrand, Dijon, France. 8. Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France; Service de rhumatologie, Centre Hospitalier Universitaire de Strasbourg, 1 avenue Molière, 67098 Strasbourg, France; Immuno-rhumatologie moléculaire, INSERM UMR-S 1109, Strasbourg, France. Electronic address: laurent.arnaud@chru-strasbourg.fr.
Abstract
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.
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.
Authors: Pil Gyu Park; Jung Yoon Pyo; Sung Soo Ahn; Hyun Joon Choi; Jason Jungsik Song; Yong-Beom Park; Ji Hye Huh; Sang-Won Lee Journal: Front Cardiovasc Med Date: 2022-06-23
Authors: Caitrin M Coffey; Yasser A Radwan; Avneek S Sandhu; Cynthia S Crowson; Philippe R Bauer; Eric L Matteson; Ashima Makol Journal: J Scleroderma Relat Disord Date: 2021-06-23