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Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection

BACKGROUND: Adult-onset Still’s disease (AOSD) is a multi-system, auto-inflammatory disease characterized by fever, arthralgia, typical rash, leukocytosis, sore throat, and liver dysfunction, among other symptoms. Retrospective studies about the frequencies of AOSD have shown that this disease is ve...

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Autores principales: Gottschalk, Maxime N., Heiland, Max, Nahles, Susanne, Preissner, Robert, Petri, William A., Wendy, Stephanie, Preissner, Saskia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999054/
https://www.ncbi.nlm.nih.gov/pubmed/36899416
http://dx.doi.org/10.1186/s13023-023-02651-3
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author Gottschalk, Maxime N.
Heiland, Max
Nahles, Susanne
Preissner, Robert
Petri, William A.
Wendy, Stephanie
Preissner, Saskia
author_facet Gottschalk, Maxime N.
Heiland, Max
Nahles, Susanne
Preissner, Robert
Petri, William A.
Wendy, Stephanie
Preissner, Saskia
author_sort Gottschalk, Maxime N.
collection PubMed
description BACKGROUND: Adult-onset Still’s disease (AOSD) is a multi-system, auto-inflammatory disease characterized by fever, arthralgia, typical rash, leukocytosis, sore throat, and liver dysfunction, among other symptoms. Retrospective studies about the frequencies of AOSD have shown that this disease is very rare. However, there has been an increased scientific interest in the last 2 years, as numerous case studies on AOSD have been published. These case studies describe the occurrence of AOSD after SARS-CoV-2 infection and/or COVID-19 vaccination. METHODS: We analyzed the incidence of AOSD to examine a potential association between AOSD and SARS-CoV-2 infection and/or COVID-19 vaccination. The TriNetX dataset consists of 90 million patients. We found 8474 AOSD cases, which we analyzed regarding SARS-CoV-2 infection and/or vaccination status. We also analyzed the cohorts considering demographic data, lab values, co-diagnoses and treatment pathways. RESULTS: We divided the AOSD cases into four cohorts: primary cohort (AOSD), Cov cohort (AOSD + SARS-CoV-2 infection), Vac cohort (AOSD + COVID-19 vaccination) and Vac + Cov cohort (AOSD + COVID-19 vaccination + SARS-CoV-2 infection). For the primary cohort, we found an annual incidence of 0.35 per 100.000. We found an association between AOSD and SARS-CoV-2 infection and/or COVID-19 vaccination. According to the numerical analysis, the incidence of AOSD doubled for the Cov cohort and Vac cohort. Moreover, the incidence of AOSD was 4.82 times higher for Vac + Cov cohort. The lab values for inflammatory markers were increased. Co-diagnoses such as rash, sore throat, and fever appeared in all AOSD cohorts, with the highest occurrences in the AOSD + COVID-19 vaccination + SARS-CoV-2 infection cohort. We identified several lines of treatments, mainly in association with adrenal corticosteroids. CONCLUSIONS: This research supports the assumption of an association between AOSD and SARS-CoV-2 infection and/or COVID-19 vaccination. However, AOSD remains a rare disease and the usage of vaccines to fight the COVID-19 pandemic should not be questioned because of the increased incidence of AOSD. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-023-02651-3.
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spelling pubmed-99990542023-03-10 Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection Gottschalk, Maxime N. Heiland, Max Nahles, Susanne Preissner, Robert Petri, William A. Wendy, Stephanie Preissner, Saskia Orphanet J Rare Dis Research BACKGROUND: Adult-onset Still’s disease (AOSD) is a multi-system, auto-inflammatory disease characterized by fever, arthralgia, typical rash, leukocytosis, sore throat, and liver dysfunction, among other symptoms. Retrospective studies about the frequencies of AOSD have shown that this disease is very rare. However, there has been an increased scientific interest in the last 2 years, as numerous case studies on AOSD have been published. These case studies describe the occurrence of AOSD after SARS-CoV-2 infection and/or COVID-19 vaccination. METHODS: We analyzed the incidence of AOSD to examine a potential association between AOSD and SARS-CoV-2 infection and/or COVID-19 vaccination. The TriNetX dataset consists of 90 million patients. We found 8474 AOSD cases, which we analyzed regarding SARS-CoV-2 infection and/or vaccination status. We also analyzed the cohorts considering demographic data, lab values, co-diagnoses and treatment pathways. RESULTS: We divided the AOSD cases into four cohorts: primary cohort (AOSD), Cov cohort (AOSD + SARS-CoV-2 infection), Vac cohort (AOSD + COVID-19 vaccination) and Vac + Cov cohort (AOSD + COVID-19 vaccination + SARS-CoV-2 infection). For the primary cohort, we found an annual incidence of 0.35 per 100.000. We found an association between AOSD and SARS-CoV-2 infection and/or COVID-19 vaccination. According to the numerical analysis, the incidence of AOSD doubled for the Cov cohort and Vac cohort. Moreover, the incidence of AOSD was 4.82 times higher for Vac + Cov cohort. The lab values for inflammatory markers were increased. Co-diagnoses such as rash, sore throat, and fever appeared in all AOSD cohorts, with the highest occurrences in the AOSD + COVID-19 vaccination + SARS-CoV-2 infection cohort. We identified several lines of treatments, mainly in association with adrenal corticosteroids. CONCLUSIONS: This research supports the assumption of an association between AOSD and SARS-CoV-2 infection and/or COVID-19 vaccination. However, AOSD remains a rare disease and the usage of vaccines to fight the COVID-19 pandemic should not be questioned because of the increased incidence of AOSD. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-023-02651-3. BioMed Central 2023-03-10 /pmc/articles/PMC9999054/ /pubmed/36899416 http://dx.doi.org/10.1186/s13023-023-02651-3 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Gottschalk, Maxime N.
Heiland, Max
Nahles, Susanne
Preissner, Robert
Petri, William A.
Wendy, Stephanie
Preissner, Saskia
Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection
title Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection
title_full Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection
title_fullStr Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection
title_full_unstemmed Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection
title_short Increased incidence of adult-onset Still’s disease in association with COVID-19 vaccination and SARS-CoV-2 infection
title_sort increased incidence of adult-onset still’s disease in association with covid-19 vaccination and sars-cov-2 infection
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999054/
https://www.ncbi.nlm.nih.gov/pubmed/36899416
http://dx.doi.org/10.1186/s13023-023-02651-3
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