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BMC Medicine
Published in: BMC Medicine 1/2016

Open Access 01-12-2016 | Research article

Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

Authors: Piero Ruscitti, Paola Cipriani, Francesco Masedu, Daniela Iacono, Francesco Ciccia, Vasiliki Liakouli, Giuliana Guggino, Francesco Carubbi, Onorina Berardicurti, Paola Di Benedetto, Marco Valenti, Giovanni Triolo, Gabriele Valentini, Roberto Giacomelli

Published in: BMC Medicine | Issue 1/2016

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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.
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Metadata
Title
Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers
Authors
Piero Ruscitti
Paola Cipriani
Francesco Masedu
Daniela Iacono
Francesco Ciccia
Vasiliki Liakouli
Giuliana Guggino
Francesco Carubbi
Onorina Berardicurti
Paola Di Benedetto
Marco Valenti
Giovanni Triolo
Gabriele Valentini
Roberto Giacomelli
Publication date
01-12-2016
Publisher
BioMed Central
Published in
BMC Medicine / Issue 1/2016
Electronic ISSN: 1741-7015
DOI
https://doi.org/10.1186/s12916-016-0738-8

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