Ruscitti Piero, Masedu Francesco, Vitale Antonio, Caggiano Valeria, Di Cola Ilenia, Cipriani Paola, Valenti Marco, Mayrink Giardini Henrique A, de Brito Antonelli Isabele Parente, Dagostin Marilia Ambiel, Lopalco Giuseppe, Iannone Florenzo, Maria Morrone, Almaghlouth Ibrahim A, Asfina Kazi Nur, Ali Hebatallah Hamed, Ciccia Francesco, Iacono Daniela, Pantano Ilenia, Mauro Daniele, Sfikakis Petros P, Tektonidou Maria, Laskari Katerina, Berardicurti Onorina, Dagna Lorenzo, Tomelleri Alessandro, Tufan Abdurrahman, Can Kardas Rıza, Hinojosa-Azaola Andrea, Martín-Nares Eduardo, Kawakami-Campos Perla Ayumi, Ragab Gaafar, Hegazy Mohamed Tharwat, Direskeneli Haner, Alibaz-Oner Fatma, Fotis Lampros, Sfriso Paolo, Govoni Marcello, La Torre Francesco, Cristina Maggio Maria, Montecucco Carlomaurizio, De Stefano Ludovico, Bugatti Serena, Rossi Silvia, Makowska Joanna, Del Giudice Emanuela, Emmi Giacomo, Bartoloni Elena, Hernández-Rodríguez José, Conti Giovanni, Nunzia Olivieri Alma, Lo Gullo Alberto, Simonini Gabriele, Viapiana Ombretta, Wiesik-Szewczyk Ewa, Erten Sukran, Carubbi Francesco, De Paulis Amato, Maier Armin, Tharwat Samar, Costi Stefania, Iagnocco Annamaria, Sebastiani Gian Domenico, Gidaro Antonio, Brucato Antonio Luca, Karamanakos Anastasios, Akkoç Nurullah, Caso Francesco, Costa Luisa, Prete Marcella, Perosa Federico, Atzeni Fabiola, Guggino Giuliana, Fabiani Claudia, Frediani Bruno, Giacomelli Roberto, Cantarini Luca
Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Arthritis Rheumatol. 2024 Jul;76(7):1141-1152. doi: 10.1002/art.42845. Epub 2024 May 15.
We aimed to evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly.
A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis.
A total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07-1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81-6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48-2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14-4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations.
The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.
我们旨在评估系统评分在预测斯蒂尔病危及生命进展方面的临床实用性。我们还旨在评估系统评分各组成部分在预测危及生命进展方面的临床相关性，并据此得出患者亚组。
设计了一项多中心、观察性、前瞻性研究，纳入意大利临床与实验风湿病学研究组成人起病斯蒂尔病研究组以及自身炎症性疾病联盟网络斯蒂尔病登记处的患者。对患者进行评估，以确定是否可获得用于计算系统评分的变量。危及生命的进展定义为无论临床病程如何的死亡，和/或巨噬细胞活化综合征，一种与预后不良相关的继发性噬血细胞性淋巴组织细胞增生症。
共评估了597例斯蒂尔病患者（平均±标准差年龄36.6±17.3岁；男性占44.4%）。作为连续变量评估的系统评分显著预测了危及生命的进展（比值比[OR]1.24；95%置信区间[CI]1.07 - 1.42；P = 0.004）。系统评分≥7也显著预测了患者经历危及生命进展的可能性（OR 3.36；95% CI 1.81 - 6.25；P < 0.001）。评估系统评分各组成部分的临床相关性时，肝脏受累（OR 1.68；95% CI 1.48 - 2.67；P = 0.031）和肺部疾病（OR 2.12；95% CI 1.14 - 4.49；P = 0.042）均显著预测了危及生命的进展。得出了肝脏受累和肺部疾病患者的临床特征，突出了它们在多器官疾病表现中的相关性。
在一大群患者中，系统评分的临床实用性体现在识别出具有更高危及生命进展风险的斯蒂尔病患者。此外，突出了肝脏受累和肺部疾病的临床相关性。
