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Latent class analysis of 216 patients with adult-onset Still’s disease
BACKGROUND: Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease which encompasses patients with heterogenous presentation and a wide range of clinical courses. In this study, we aimed to identify potential subgroups of AOSD and reveal risk factors for relapse. METHODS: We...
Autores principales: | , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8722082/ https://www.ncbi.nlm.nih.gov/pubmed/34980244 http://dx.doi.org/10.1186/s13075-021-02708-3 |
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author | Sugiyama, Takahiro Furuta, Shunsuke Hiraguri, Masaki Ikeda, Kei Inaba, Yosuke Kagami, Shin-ichiro Kita, Yasuhiko Kobayashi, Kei Kobayashi, Yoshihisa Kurasawa, Kazuhiro Nakagomi, Daiki Nawata, Yasushi Kawasaki, Yohei Shiko, Yuki Sugiyama, Takao Nakajima, Hiroshi |
author_facet | Sugiyama, Takahiro Furuta, Shunsuke Hiraguri, Masaki Ikeda, Kei Inaba, Yosuke Kagami, Shin-ichiro Kita, Yasuhiko Kobayashi, Kei Kobayashi, Yoshihisa Kurasawa, Kazuhiro Nakagomi, Daiki Nawata, Yasushi Kawasaki, Yohei Shiko, Yuki Sugiyama, Takao Nakajima, Hiroshi |
author_sort | Sugiyama, Takahiro |
collection | PubMed |
description | BACKGROUND: Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease which encompasses patients with heterogenous presentation and a wide range of clinical courses. In this study, we aimed to identify potential subgroups of AOSD and reveal risk factors for relapse. METHODS: We included a total of 216 AOSD patients who received treatment in nine hospitals between 2000 and 2019. All patients fulfilled the Yamaguchi classification criteria. We retrospectively collected information about baseline characteristics, laboratory tests, treatment, relapse, and death. We performed latent class analysis and time-to-event analysis for relapse using the Cox proportional hazard model. RESULTS: The median age at disease onset was 51.6 years. The median follow-up period was 36.8 months. At disease onset, 22.3% of the patients had macrophage activation syndrome. The median white blood cell count was 12,600/μL, and the median serum ferritin level was 7230 ng/mL. Systemic corticosteroids were administered in all but three patients (98.6%) and the median initial dosage of prednisolone was 40mg/day. Ninety-six patients (44.4%) were treated with concomitant immunosuppressants, and 22 (10.2%) were treated with biologics. Latent class analysis revealed that AOSD patients were divided into two subgroups: the typical group (Class 1: 71.8%) and the elderly-onset group (Class 2: 28.2%). During the follow-up period, 13 of 216 patients (6.0%) died (12 infections and one senility), and 76 of 216 patients (35.1%) experienced relapses. Overall and relapse-free survival rates at 5 years were 94.9% and 57.3%, respectively, and those rates were not significantly different between Class 1 and 2 (p=0.30 and p=0.19). Time-to-event analysis suggested higher neutrophil count, lower hemoglobin, and age ≥65 years at disease onset as risk factors for death and age ≥65 years at disease onset as a risk factor for relapse. CONCLUSIONS: AOSD patients were divided into two subgroups: the typical group and the elderly-onset group. Although the survival of patients with AOSD was generally good, the patients often experienced relapses. Age ≥65 years at disease onset was the risk factor for relapse. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13075-021-02708-3. |
format | Online Article Text |
id | pubmed-8722082 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-87220822022-01-06 Latent class analysis of 216 patients with adult-onset Still’s disease Sugiyama, Takahiro Furuta, Shunsuke Hiraguri, Masaki Ikeda, Kei Inaba, Yosuke Kagami, Shin-ichiro Kita, Yasuhiko Kobayashi, Kei Kobayashi, Yoshihisa Kurasawa, Kazuhiro Nakagomi, Daiki Nawata, Yasushi Kawasaki, Yohei Shiko, Yuki Sugiyama, Takao Nakajima, Hiroshi Arthritis Res Ther Research Article BACKGROUND: Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease which encompasses patients with heterogenous presentation and a wide range of clinical courses. In this study, we aimed to identify potential subgroups of AOSD and reveal risk factors for relapse. METHODS: We included a total of 216 AOSD patients who received treatment in nine hospitals between 2000 and 2019. All patients fulfilled the Yamaguchi classification criteria. We retrospectively collected information about baseline characteristics, laboratory tests, treatment, relapse, and death. We performed latent class analysis and time-to-event analysis for relapse using the Cox proportional hazard model. RESULTS: The median age at disease onset was 51.6 years. The median follow-up period was 36.8 months. At disease onset, 22.3% of the patients had macrophage activation syndrome. The median white blood cell count was 12,600/μL, and the median serum ferritin level was 7230 ng/mL. Systemic corticosteroids were administered in all but three patients (98.6%) and the median initial dosage of prednisolone was 40mg/day. Ninety-six patients (44.4%) were treated with concomitant immunosuppressants, and 22 (10.2%) were treated with biologics. Latent class analysis revealed that AOSD patients were divided into two subgroups: the typical group (Class 1: 71.8%) and the elderly-onset group (Class 2: 28.2%). During the follow-up period, 13 of 216 patients (6.0%) died (12 infections and one senility), and 76 of 216 patients (35.1%) experienced relapses. Overall and relapse-free survival rates at 5 years were 94.9% and 57.3%, respectively, and those rates were not significantly different between Class 1 and 2 (p=0.30 and p=0.19). Time-to-event analysis suggested higher neutrophil count, lower hemoglobin, and age ≥65 years at disease onset as risk factors for death and age ≥65 years at disease onset as a risk factor for relapse. CONCLUSIONS: AOSD patients were divided into two subgroups: the typical group and the elderly-onset group. Although the survival of patients with AOSD was generally good, the patients often experienced relapses. Age ≥65 years at disease onset was the risk factor for relapse. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13075-021-02708-3. BioMed Central 2022-01-03 2022 /pmc/articles/PMC8722082/ /pubmed/34980244 http://dx.doi.org/10.1186/s13075-021-02708-3 Text en © The Author(s) 2021 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 Article Sugiyama, Takahiro Furuta, Shunsuke Hiraguri, Masaki Ikeda, Kei Inaba, Yosuke Kagami, Shin-ichiro Kita, Yasuhiko Kobayashi, Kei Kobayashi, Yoshihisa Kurasawa, Kazuhiro Nakagomi, Daiki Nawata, Yasushi Kawasaki, Yohei Shiko, Yuki Sugiyama, Takao Nakajima, Hiroshi Latent class analysis of 216 patients with adult-onset Still’s disease |
title | Latent class analysis of 216 patients with adult-onset Still’s disease |
title_full | Latent class analysis of 216 patients with adult-onset Still’s disease |
title_fullStr | Latent class analysis of 216 patients with adult-onset Still’s disease |
title_full_unstemmed | Latent class analysis of 216 patients with adult-onset Still’s disease |
title_short | Latent class analysis of 216 patients with adult-onset Still’s disease |
title_sort | latent class analysis of 216 patients with adult-onset still’s disease |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8722082/ https://www.ncbi.nlm.nih.gov/pubmed/34980244 http://dx.doi.org/10.1186/s13075-021-02708-3 |
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