The Systemic Score May Identify Life‐Threatening Evolution in Still Disease: Data from the GIRRCS AOSD‐Study Group and the AIDA Network Still Disease Registry
Authors: Piero Ruscitti, Francesco Masedu, Antonio Vitale, Valeria Caggiano, Ilenia Di Cola, Paola Cipriani, Marco Valenti, Henrique A Mayrink Giardini, Isabele Parente de Brito Antonelli, Marilia Ambiel Dagostin, Giuseppe Lopalco, Florenzo Iannone, Morrone Maria, Ibrahim A Almaghlouth, Kazi Nur Asfina, Hebatallah Hamed Ali, Francesco Ciccia, Daniela Iacono, Ilenia Pantano, Daniele Mauro, Petros P Sfikakis, Maria Tektonidou, Katerina Laskari, Onorina Berardicurti, Lorenzo Dagna, Alessandro Tomelleri, Abdurrahman Tufan, Rıza Can Kardas, Andrea Hinojosa‐Azaola, Eduardo Martín‐Nares, Perla Ayumi Kawakami‐Campos, Gaafar Ragab, Mohamed Tharwat Hegazy, Haner Direskeneli, Fatma Alibaz‐Oner, Lampros Fotis, Paolo Sfriso, Marcello Govoni, Francesco La Torre, Maria Cristina Maggio, Carlomaurizio Montecucco, Ludovico De Stefano, Serena Bugatti, Silvia Rossi, Joanna Makowska, Emanuela Del Giudice, Giacomo Emmi, Elena Bartoloni, José Hernández‐Rodríguez, Giovanni Conti, Alma Nunzia Olivieri, Alberto Lo Gullo, Gabriele Simonini, Ombretta Viapiana, Ewa Wiesik‐Szewczyk, Sukran Erten, Francesco Carubbi, Amato De Paulis, Armin Maier, Samar Tharwat, Stefania Costi, Annamaria Iagnocco, Gian Domenico Sebastiani, Antonio Gidaro, Antonio Luca Brucato, Anastasios Karamanakos, Nurullah Akkoç, Francesco Caso, Luisa Costa, Marcella Prete, Federico Perosa, Fabiola Atzeni, Giuliana Guggino, Claudia Fabiani, Bruno Frediani, Roberto Giacomelli, Luca Cantarini
Journal: Arthritis & Rheumatology (2024)

Objective 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. Methods 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. Results 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 &lt; 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. Conclusion 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. image