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Macrophage activation syndrome in Still’s disease: analysis of clinical characteristics and survival in paediatric and adult patients

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Abstract

Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, complicating Still’s disease, both in paediatric and adult patients. In this work, we aimed to investigate clinical picture and outcome of Still’s disease patients developing MAS. We performed a retrospective analysis of patients, both paediatrics and adults, affected by Still’s disease attending our department. During the follow-up, each patient was investigated for MAS occurrence and possible predictors, clinical and laboratory factors, were analysed. We evaluated 50 patients affected by Still’s disease, 21 paediatric and 29 adult patients. Ten patients experienced MAS (five adult and five paediatric patients) and its development significantly reduced the survival rate when compared with patients without this complication (p < 0.0001). The analysis of possible predictors showed that high-value systemic score (p = 0.03) and high levels of serum ferritin (p = 0.002) were independently associated with an increased likelihood of MAS. MAS occurrence significantly reduced survival rate in both paediatric and adult patients affected by Still’s disease. The high levels of serum ferritin and an elevated systemic score, at the time of diagnosis, were significantly associated with MAS.

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Acknowledgements

The authors thank Mrs. Federica Sensini for her technical assistance.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, L’Aquila, Italy

    Piero Ruscitti, Paola Cipriani, Vasiliki Liakouli, Onorina Berardicurti, Francesco Carubbi & Roberto Giacomelli

  2. Department of Paediatrics, University of L’Aquila, L’Aquila, Italy

    Carmela Rago & Alberto Verrotti

  3. Department of Paediatrics, University of Chieti, Chieti, Italy

    Luciana Breda & Caterina Di Battista

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  1. Piero Ruscitti
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  2. Carmela Rago
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  3. Luciana Breda
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  4. Paola Cipriani
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  5. Vasiliki Liakouli
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  10. Roberto Giacomelli
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Correspondence to Piero Ruscitti.

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Ruscitti, P., Rago, C., Breda, L. et al. Macrophage activation syndrome in Still’s disease: analysis of clinical characteristics and survival in paediatric and adult patients. Clin Rheumatol 36, 2839–2845 (2017). https://doi.org/10.1007/s10067-017-3830-3

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  • DOI: https://doi.org/10.1007/s10067-017-3830-3

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