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

Francesco Ciccia, Giuliana Guggino, Vasiliki Liakouli, Francesco Carubbi, Paola Di Benedetto, Piero Ruscitti, Onorina Berardicurti, Salvatore Di Bartolomeo, Francesco Masedu, Roberto Giacomelli, Paola Cipriani, Marco Valenti

Research output: Contribution to journalArticlepeer-review

38 Citations (Scopus)

Abstract

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.
Original languageEnglish
Pages (from-to)16-21
Number of pages6
JournalAutoimmunity Reviews
Volume16
Publication statusPublished - 2016

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology

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