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

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BMC Medicine

, 14:194

First Online: 01 December 2016Received: 16 July 2016Accepted: 03 November 2016DOI: 10.1186-s12916-016-0738-8

Cite this article as: Ruscitti, P., Cipriani, P., Masedu, F. et al. BMC Med 2016 14: 194. doi:10.1186-s12916-016-0738-8


BackgroundAdult-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.

MethodsThis 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.

ResultsOne 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.

ConclusionsOur 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.

KeywordsAdult-onset Still’s disease Systemic score Macrophage activation syndrome Prognostic factor AbbreviationsAOSDadult-onset Still’s disease

CRPC-reactive protein

ESRerythrocyte sedimentation rate


MASmacrophage activation syndrome


sDMARDssynthetic disease-modifying anti-rheumatic drugs

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Autor: Piero Ruscitti - Paola Cipriani - Francesco Masedu - Daniela Iacono - Francesco Ciccia - Vasiliki Liakouli - Giuliana Guggi


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