Abstract
ObjectivesTo define different clinical phenotypes and assess prognostic factors of adult-onset Still’s disease (AOSD).MethodsOverall, 492 patients with AOSD seen between 2004 and 2018 at a single centre were retrospectively studied.ResultsOf 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).ConclusionThree 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.
Highlights
Adult-onset Still’s disease (AOSD) is a systemic inflammatory disorder of unknown aetiology
The lack of distinguished clinical phenotypes based on the clinical features present at the time of diagnosis as well as the exact report of the long-term survival rate for AOSD based on a large sample size has slowed the progress of precision management of AOSD
A monocyclic pattern was defined as a single episode throughout the entire follow-up period; a relapsing pattern was defined as multiple flares of systemic and/or articular symptoms alternating with disease-free intervals; and a chronic pattern was defined as having persistent symptoms, such as polyarthritis [21]
Summary
Adult-onset Still’s disease (AOSD) is a systemic inflammatory disorder of unknown aetiology. Three clinical patterns with acceptable clinical significance [1, 8, 10], i.e., the monocyclic pattern, the polycyclic pattern, and the chronic pattern, are generally identified according to the disease course [11]. This classification is not directly based on the clinical features and provides limited information on the management of new-onset AOSD. A better understanding and management of the disease would be possible if the potential clinical models of AOSD were further differentiated
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