title: Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. pieroruscitti@live.com.
authors.forename: Piero
authors.initials: P
authors.lastname: Ruscitti
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Cipriani
authors.affiliation: Medical Statistic Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Masedu
authors.affiliation: Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Iacono
authors.affiliation: Rheumatology Section, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Ciccia
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Vasiliki
authors.initials: V
authors.lastname: Liakouli
authors.affiliation: Rheumatology Section, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Guggino
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Carubbi
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Onorina
authors.initials: O
authors.lastname: Berardicurti
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Di Benedetto
authors.affiliation: Medical Statistic Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Valenti
authors.affiliation: Rheumatology Section, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Triolo
authors.affiliation: Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.affiliation: Rheumatology Section, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Giacomelli
mesh_terms: D000328:Adult; D015415:Biomarkers; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000175:diagnosis* / Q000401:mortality*; D055815:Young Adult
chemical_list: D015415:Biomarkers
keywords: Adult-onset Still’s disease; Macrophage activation syndrome; Prognostic factor; Systemic score