Clinical phenotypes and prognostic factors of adult-onset Still's disease: data from a large inpatient cohort.
Clinical phenotypes and prognostic factors of adult-onset Still's disease: data from a large inpatient cohort.
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DOI：
10.1186/s13075-021-02688-4
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发表时间：
2021-12-08
影响因子：
4.9
通讯作者：
Lu L
中科院分区：
文献类型：
--
作者：
Li R;Liu X;Chen G;Tang G;Chen X;Liu X;Wang J;Lu L
目的明确成人斯蒂尔病（AOSD）的不同临床表型并评估其预后因素。总体而言，回顾性研究了2004年至2018年在一个中心观察的492例AOSD患者。在这些患者中，78%为女性，发病时的中位年龄为34（25-49）岁[中位数（第25 - 75百分位数）]。中位随访时间为7（3-10）年[中位数（第25 - 75百分位数）]。根据入院时的临床表现将AOSD患者细分为：全身性炎症（第1组）、单纯性炎症（第2组）和中间性炎症（第3组）。每个亚型有不同的临床表现和症状：第1组（34.6%）-多器官表现，感染率和死亡率最高，超过一半的患者在随访期间至少复发一次;第2组（21.3%）-仅女性，皮疹和关节受累，无内脏器官受累，无死亡，大多数患者为单周期病程;组3（44.1%）-感染率低，无严重并发症，死亡率低。确诊后5年和10年生存率分别为92.4%和86.9%。死亡的独立危险因素为发病年龄≥50岁（风险比（HR）：6.78，95% CI：2.10-21.89），肝肿大（HR：5.05，95% CI：1.44-17.70）、感染（HR：15.56，95% CI：5.88-41.20）和MAS（HR：26.82，95% CI：7.52-95.60）。AOSD分为3个亚型，临床表现和体征各不相同。发病年龄≥50岁、肝肿大、感染和MAS是AOSD死亡率的预后因素。在线版本包含补充材料，可通过10.1186/s13075-021-02688-4获得。
To define different clinical phenotypes and assess prognostic factors of adult-onset Still’s disease (AOSD). Overall, 492 patients with AOSD seen between 2004 and 2018 at a single centre were retrospectively studied. 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). 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. The online version contains supplementary material available at 10.1186/s13075-021-02688-4.
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影响因子：
--
作者：
Franchini, Stefano;Dagna, Lorenzo;Sabbadini, Maria Grazia
通讯作者：
Sabbadini, Maria Grazia
影响因子：
2.1
作者：
Nossent, HC
通讯作者：
Nossent, HC
影响因子：
13.3
作者：
Fardet, Laurence;Galicier, Lionel;Hejblum, Gilles
通讯作者：
Hejblum, Gilles
影响因子：
9.3
作者：
Ruscitti P;Cipriani P;Masedu F;Iacono D;Ciccia F;Liakouli V;Guggino G;Carubbi F;Berardicurti O;Di Benedetto P;Valenti M;Triolo G;Valentini G;Giacomelli R
通讯作者：
Giacomelli R
影响因子：
8.6
作者：
Inoue, Natsumi;Shimizu, Masaki;Yachie, Akihiro
通讯作者：
Yachie, Akihiro