Latent class analysis of 216 patients with adult-onset Still's disease.
Adult-onset Still’s disease
Latent class analysis
Macrophage activation syndrome
Outcomes
Relapse
Journal
Arthritis research & therapy
ISSN: 1478-6362
Titre abrégé: Arthritis Res Ther
Pays: England
ID NLM: 101154438
Informations de publication
Date de publication:
03 01 2022
03 01 2022
Historique:
received:
16
12
2020
accepted:
17
12
2021
entrez:
4
1
2022
pubmed:
5
1
2022
medline:
11
3
2022
Statut:
epublish
Résumé
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. 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. 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. 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.
Sections du résumé
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.
Identifiants
pubmed: 34980244
doi: 10.1186/s13075-021-02708-3
pii: 10.1186/s13075-021-02708-3
pmc: PMC8722082
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
7Commentaires et corrections
Type : CommentIn
Informations de copyright
© 2021. The Author(s).
Références
Rheumatology (Oxford). 2020 Jul 1;59(7):1725-1733
pubmed: 31965185
Rheumatol Int. 2016 Oct;36(10):1399-405
pubmed: 27502500
Medicine (Baltimore). 2015 Jan;94(4):e451
pubmed: 25634183
Arthritis Rheumatol. 2014 Aug;66(8):2297-307
pubmed: 24756912
Clin Rheumatol. 2010 Sep;29(9):1015-9
pubmed: 20549276
Mod Rheumatol. 2015 May;25(3):393-400
pubmed: 25382730
Clin Rheumatol. 2020 Aug;39(8):2379-2386
pubmed: 32130578
Arthritis Care Res (Hoboken). 2013 May;65(5):822-6
pubmed: 23225779
Semin Arthritis Rheum. 2011 Oct;41(2):279-85
pubmed: 21377714
Am J Med Sci. 2009 May;337(5):373-6
pubmed: 19322066
Ann Rheum Dis. 1971 Mar;30(2):121-33
pubmed: 5315135
South Med J. 2003 Jan;96(1):46-9
pubmed: 12602713
Rheumatology (Oxford). 2021 Oct 2;60(10):4844-4849
pubmed: 33404641
Blood. 2004 Jun 1;103(11):4043-9
pubmed: 14982878
Med Chir Trans. 1897;80:47-60.9
pubmed: 20896907
Pediatr Blood Cancer. 2007 Feb;48(2):124-31
pubmed: 16937360
Am J Med. 1995 Apr;98(4):384-8
pubmed: 7709952
Mod Rheumatol. 2014 Jul;24(4):645-50
pubmed: 24252024
BMC Med. 2013 Aug 22;11:185
pubmed: 23968282
Clin Rheumatol. 2006 Sep;25(5):639-44
pubmed: 16365690
Medicine (Baltimore). 1991 Mar;70(2):118-36
pubmed: 2005777
Curr Opin Pulm Med. 1999 Sep;5(5):305-9
pubmed: 10461535
Ann Rheum Dis. 2006 Dec;65(12):1596-601
pubmed: 16540551
Int J Clin Pract. 2009 Jul;63(7):1050-5
pubmed: 17511792
Lancet. 2014 Apr 26;383(9927):1503-1516
pubmed: 24290661
Ann Rheum Dis. 2020 Aug;79(8):1090-1097
pubmed: 32404342
Rheumatol Int. 2012 May;32(5):1291-8
pubmed: 21274538
Autoimmun Rev. 2014 Jul;13(7):708-22
pubmed: 24657513
Clin Rheumatol. 2016 May;35(5):1377-82
pubmed: 24737284
J Rheumatol. 2018 Jun;45(6):864-872
pubmed: 29657144
Arthritis Rheum. 1987 Feb;30(2):186-94
pubmed: 3827959
Hematology Am Soc Hematol Educ Program. 2009;:127-31
pubmed: 20008190
Ann Rheum Dis. 2019 Dec;78(12):e133
pubmed: 30389689
BMC Med. 2016 Dec 1;14(1):194
pubmed: 27903264
Clin Rheumatol. 2014 Mar;33(3):305-14
pubmed: 24435354
Clin Exp Rheumatol. 2014 Jan-Feb;32(1):28-33
pubmed: 24050706
Immunol Res. 2015 Feb;61(1-2):53-62
pubmed: 25388963
Autoimmun Rev. 2014 Nov;13(11):1149-59
pubmed: 25183244
J Rheumatol. 1992 Mar;19(3):424-30
pubmed: 1578458
Semin Arthritis Rheum. 2019 Dec;49(3):469-473
pubmed: 31109638
Arthritis Rheumatol. 2014 Sep;66(9):2613-20
pubmed: 24782338
Semin Oncol. 1991 Feb;18(1):29-33
pubmed: 1992521
Struct Equ Modeling. 2007;14(4):671-694
pubmed: 19953201
J Rheumatol. 2001 Feb;28(2):322-9
pubmed: 11246670