Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers.
Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers.
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DOI：
10.1186/s12916-016-0738-8
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发表时间：
2016-12-01
期刊：
影响因子：
9.3
通讯作者：
Giacomelli R
中科院分区：
文献类型：
--
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
成人斯蒂尔病（AOSD）是一种罕见的炎性疾病，病因不明，通常影响年轻人。较常见的临床表现是尖峰热、关节炎、一过性皮疹、肝酶升高、淋巴结病、肝脾肿大和浆膜炎。该病的多脏器受累和不同的并发症，如巨噬细胞活化综合征，可能会大大降低AOSD患者的预期寿命。本研究旨在确定与患者结局相关的积极和消极特征。对三个风湿病中心前瞻性收治的AOSD患者进行回顾性分析，以确定诊断时的临床特征并预测可能的结果。此外，我们还研究了尚未得到验证的预后价值的系统评分先前提出的。入组了100例连续的AOSD患者。平均全身评分显示，大多数患者有多器官受累。16例患者出现不同的并发症，主要是巨噬细胞活化综合征。观察到炎症标志物的强烈增加。所有患者均接受不同剂量的类固醇治疗，55例患者与免疫抑制药物联合治疗，32例患者与生物制剂联合治疗。16例患者在随访期间死亡。回归分析显示，诊断时评估的较高的系统评分值和AOSD相关并发症的存在与患者死亡率显著相关。报告了系统评分≥ 7.0的预后影响。我们的研究表明，较高的系统评分和诊断时存在AOSD相关并发症与死亡率显著相关。值得注意的是，系统评分7.0的临界值显示出对确定存在AOSD相关死亡风险的患者具有很强的预后影响。
Adult-onset Still’s disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients. This study aimed to identify the positive and negative features correlated with the outcome of patients. A retrospective analysis of AOSD patients prospectively admitted to three rheumatologic centers was performed to identify the clinical features present at the time of diagnosis and to predict the possible outcome. Furthermore, we investigated the as yet to be validated prognostic value of the systemic score previously proposed. One hundred consecutive AOSD patients were enrolled. The mean systemic score showed that the majority of patients had a multi-organ involvement. Sixteen patients showed different complications, mainly the macrophage activation syndrome. A strong increase of inflammatory markers was observed. All patients received steroids at different dosages, 55 patients in association with immunosuppressive drugs and 32 in association with biologic agents. Sixteen patients died during the follow-up. Regression analysis showed that the higher values of the systemic score and the presence of AOSD-related complications, assessed at the time of diagnosis, were significantly correlated with patient mortality. A prognostic impact of the systemic score of ≥ 7.0 was reported. Our study showed that a higher systemic score and the presence of AOSD-related complications at the time of diagnosis were significantly associated with mortality. Of note, a cut-off at 7.0 of the systemic score showed a strong prognostic impact in identifying patients at risk of AOSD-related death.
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影响因子：
2.8
作者：
Jamilloux Y;Gerfaud-Valentin M;Henry T;Sève P
通讯作者：
Sève P
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
作者：
Giampietro C;Fautrel B
通讯作者：
Fautrel B
影响因子：
5.5
作者：
Gerards, AH;de Lathouder, S;Aarden, LA
通讯作者：
Aarden, LA
影响因子：
4.4
作者：
Jamilloux, Yvan;Gerfaud-Valentin, Mathieu;Seve, Pascal
通讯作者：
Seve, Pascal