Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 cases collected in 2 rheumatologic centers

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• 作者：Piero Ruscitti^a ; Paola Cipriani^a ; Francesco Ciccia^b ; Francesco Masedu^c ; Vasiliki Liakouli^a ; Francesco Carubbi^a ; Onorina Berardicurti^a ; Giuliana Guggino^b ; Paola Di Benedetto^a ; Salvatore Di Bartolomeo^a ; Marco Valenti^c ; Giovanni Triolo^b ; Roberto Giacomelli^a ; ^{roberto.giacomelli@cc.univaq.it}
• 关键词：Macrophage activation syndrome ; Adult onset Still's disease ; Hyperferritinemic syndrome
• 刊名：Autoimmunity Reviews
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：16
• 期：1
• 页码：16-21
• 全文大小：269 K
• 卷排序：16

文摘

Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure.In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases. MAS-related death occurred in 17 patients (42.5%) during the follow-up, and older age and increased serum ferritin levels, at the time of diagnosis, were significantly associated with mortality.In conclusion, adult MAS is associated with high mortality rate. Some clinical features at diagnosis may be predictive of MAS-associated death.