OP0001 THE EVALUATION OF SYSTEMIC SCORE IN IDENTIFYING PATIENTS WITH STILL’S DISEASE AT HIGHER RISK OF LIFE-THREATENING EVOLUTION; FINDINGS FROM GIRRCS AOSD-STUDY GROUP AND AIDA NETWORK STILL’S DISEASE REGISTRY
Authors: P. Ruscitti, A. Vitale, V. Caggiano, I. DI Cola, F. Sensini, P. Cipriani, R. Giacomelli, L. Cantarini
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Still's disease is an inflammatory disorder typically manifesting with fever, arthritis, and skin rash affecting both children and adults, formerly known as systemic juvenile idiopathic arthritis and adult-onset Still's disease, and burdened by life-threatening complications [1,2]. <h3>Objectives:</h3> To evaluate the clinical usefulness of systemic score in the prediction of life-threatening evolution in patients with Still's disease. To assess the clinical relevance of each component of the systemic score in predicting the life-threatening evolution and to derive clinical patient subset. <h3>Methods:</h3> A multicentre, observational, prospective study was built considering patients included in GIRRCS (<i>Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale</i>) AOSD-study group and AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry if specific variables for the derivation of systemic score were available assessing its predictive role on the emergence of a life-threatening evolution, defined as the development of macrophage activation syndrome (MAS) and/or mortality whichever the clinical course. <h3>Results:</h3> 597 patients with Still's disease were assessed (age 36.6 ± 17.3 years, male sex 44.4%). Among them, 84 patients had a paediatric disease onset. Patients were mainly characterised by fever (100%), joint involvement (87.9%), and skin rash (66.1%). Liver involvement was recognised in 43.5% of patients, MAS in 13.1%, and lung disease in 6.9%. A mortality rate of 3.4% was registered due to Still's disease related deaths. The systemic score resulted a significant predictor of patient life-threatening evolution OR:1.24, 95%CI:1.07-1.42, p=0.004) adjusting the model for age, male sex, inflammatory markers, and ferritin. The systemic score ≥ 7 also significantly predicted the likelihood of life-threatening evolution in assessed patients (OR:3.36, 95%CI:1.81-6.25, p&lt;0.001). The risk profile assessment was performed on the life-threatening evolution in regard to the clinical variables exploited to calculate the systemic score. An age- and male sex-adjusted multivariate logistic regression model was built accordingly; 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) independently predicted patient life-threatening evolution. After that the clinical characteristics of patients with liver involvement and lung disease were derived. Patients with liver involvement were significantly characterised by lymph-adenomegaly (OR:1.80, 95%CI: 1.29-2.51, p=0.001), splenomegaly (OR:5.37, 95%CI: 3.74-7.72, p&lt;0.0001), pericarditis (OR:2.01, 95%CI: 1.29-3.13, p=0.001) and pleuritis (OR:2.43, 95%CI: 1.55-3.80, p=0.001). Patients with liver involvement were burdened by lung disease (OR:2.15, 95%CI:1.12-4.11, p=0.018). Assessing patients with lung disease, they were significantly characterised by sore throat (OR:2.52, 95%CI:1.21-5.24, p=0.010), lymph-adenomegaly (OR:2.32, 95%CI:1.17-4.57, p=0.013), splenomegaly (OR:2.02, 95%CI:1.07-3.83, p=0.028), and liver involvement (OR:2.14, 95%CI:1.12-4.11, p=0.019). Furthermore, patients with lung disease showed an enhanced frequency of pericarditis (OR:3.74, 95%CI:1.91-7.32, p=0.001), pleuritis (OR:6.52, 95%CI:3.37-12.60, p=0.001), and abdominal pain (OR:4.13, 95%CI:2.05-8.30, p=0.001). Patients with lung disease were burdened by a higher rate of mortality (OR:3.62, 95%CI:1.15-11.41, p=0.019). <h3>Conclusion:</h3> The usefulness of systemic score was shown in recognizing those patients with Still's disease at higher risk of life-threatening evolution in a large cohort from GIRRCS AOSD-study group and AIDA Network Still's Disease Registry, thus providing a prognostic tool to be readily applied to the clinical practice. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted in regard to the multi-organ manifestations of patients and as major predictors of the life-threatening evolution. <h3>REFERENCES:</h3> [1] Lee JJY, et al. Pediatr Clin North Am. 2018;65:691-709. [2] Giacomelli R, et al. J Autoimmun. 2018;93:24-36. [3] Ruscitti P, et al. BMC Med. 2016;14:194. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>