Abstract
Abstract
Objectives
To define different clinical phenotypes and assess prognostic factors of adult-onset Still’s disease (AOSD).
Methods
Overall, 492 patients with AOSD seen between 2004 and 2018 at a single centre were retrospectively studied.
Results
Of these patients, 78% were female, and the median age at onset was 34 (25–49) years [median (25th–75th percentile)]. The median follow-up time was 7 (3–10) years [median (25th–75th percentile)]. Clinical manifestations at admission were used to subdivide patients with AOSD as follows: systemic inflammation (cluster 1), pure (cluster 2), and intermediate (cluster 3). Each subtype had distinct clinical manifestations and prognoses: cluster 1 (34.6%)—multiple organ manifestations, highest infection rate and mortality, and more than half of the patients with at least one relapse during follow-up; cluster 2 (21.3%)—exclusively female, rash and joint involvement, no internal organ involvement, no mortality, and most of the patients with a monocyclic course; and cluster 3 (44.1%)—less infection rate, no serious complications, and lower mortality rate. The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. Independent risk factors for mortality were age at onset ≥50 (hazard ratio (HR): 6.78, 95% CI: 2.10–21.89), hepatomegaly (HR: 5.05, 95% CI: 1.44–17.70), infection (HR: 15.56, 95% CI: 5.88–41.20), and MAS (HR: 26.82, 95% CI: 7.52–95.60).
Conclusion
Three subtypes of AOSD were identified with distinct clinical manifestations and prognoses. Age at onset ≥50, hepatomegaly, infection, and MAS were prognostic factors for AOSD mortality.
Funder
National Natural Science Foundation of China
Shanghai Clinical Collaboration Pilot Project of Traditional Chinese and Western Medicine
Publisher
Springer Science and Business Media LLC
Cited by
10 articles.
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