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Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort
OBJECTIVE: SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ affliction...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7516103/ https://www.ncbi.nlm.nih.gov/pubmed/32097470 http://dx.doi.org/10.1093/rheumatology/keaa026 |
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author | Fretheim, Håvard Halse, Anne-Kristine Seip, Marit Bitter, Helle Wallenius, Marianne Garen, Torhild Salberg, Anne Brunborg, Cathrine Midtvedt, Øyvind Molberg, Øyvind Hoffmann-Vold, Anna-Maria |
author_facet | Fretheim, Håvard Halse, Anne-Kristine Seip, Marit Bitter, Helle Wallenius, Marianne Garen, Torhild Salberg, Anne Brunborg, Cathrine Midtvedt, Øyvind Molberg, Øyvind Hoffmann-Vold, Anna-Maria |
author_sort | Fretheim, Håvard |
collection | PubMed |
description | OBJECTIVE: SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ afflictions in SSc. METHODS: A stepwise strategy was applied to find and characterize every SSc patient resident in Norway from 2000 to 2012. First we identified every case in the country registered with an International Classification of Diseases, Tenth Revision code for SSc (M34). Next we manually reviewed all cases coded as M34 to determine whether they met the 1980 ACR and/or 2013 ACR/EULAR classification criteria for SSc and could be included in the Norwegian SSc cohort (Nor-SSc). Finally, all disease features from SSc onset to study end were reviewed. RESULTS: The Nor-SSc cohort included 815 SSc patients. The mean age at diagnosis was 53 years, with 84% females and 77% limited cutaneous SSc. The estimated incidence increased from 4 per million in 2000 to 13 per million in 2012. We identified high cumulative frequencies of internal organ involvement, coexistence of multiple organ afflictions across disease subsets and autoantibody status and stable frequencies of pulmonary arterial hypertension across haemodynamic definitions, but indications of referral-related differences in pulmonary hypertension detection rates across the study area. CONCLUSION: This nationwide cohort study provides new, unbiased evidence for a high disease burden in SSc patients of Caucasian descent and indicates the existence of hurdles preventing equality of assessment across the SSc population. |
format | Online Article Text |
id | pubmed-7516103 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-75161032020-09-30 Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort Fretheim, Håvard Halse, Anne-Kristine Seip, Marit Bitter, Helle Wallenius, Marianne Garen, Torhild Salberg, Anne Brunborg, Cathrine Midtvedt, Øyvind Molberg, Øyvind Hoffmann-Vold, Anna-Maria Rheumatology (Oxford) Clinical Science OBJECTIVE: SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ afflictions in SSc. METHODS: A stepwise strategy was applied to find and characterize every SSc patient resident in Norway from 2000 to 2012. First we identified every case in the country registered with an International Classification of Diseases, Tenth Revision code for SSc (M34). Next we manually reviewed all cases coded as M34 to determine whether they met the 1980 ACR and/or 2013 ACR/EULAR classification criteria for SSc and could be included in the Norwegian SSc cohort (Nor-SSc). Finally, all disease features from SSc onset to study end were reviewed. RESULTS: The Nor-SSc cohort included 815 SSc patients. The mean age at diagnosis was 53 years, with 84% females and 77% limited cutaneous SSc. The estimated incidence increased from 4 per million in 2000 to 13 per million in 2012. We identified high cumulative frequencies of internal organ involvement, coexistence of multiple organ afflictions across disease subsets and autoantibody status and stable frequencies of pulmonary arterial hypertension across haemodynamic definitions, but indications of referral-related differences in pulmonary hypertension detection rates across the study area. CONCLUSION: This nationwide cohort study provides new, unbiased evidence for a high disease burden in SSc patients of Caucasian descent and indicates the existence of hurdles preventing equality of assessment across the SSc population. Oxford University Press 2020-02-25 /pmc/articles/PMC7516103/ /pubmed/32097470 http://dx.doi.org/10.1093/rheumatology/keaa026 Text en © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. http://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Clinical Science Fretheim, Håvard Halse, Anne-Kristine Seip, Marit Bitter, Helle Wallenius, Marianne Garen, Torhild Salberg, Anne Brunborg, Cathrine Midtvedt, Øyvind Molberg, Øyvind Hoffmann-Vold, Anna-Maria Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
title | Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
title_full | Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
title_fullStr | Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
title_full_unstemmed | Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
title_short | Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
title_sort | multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort |
topic | Clinical Science |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7516103/ https://www.ncbi.nlm.nih.gov/pubmed/32097470 http://dx.doi.org/10.1093/rheumatology/keaa026 |
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