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: 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 
Published: March 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 < 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.

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