Advances in immunopathogenesis of macrophage activation syndrome during rheumatic inflammatory diseases: toward new therapeutic targets?

Giovanni Triolo, Giuliana Guggino, Francesco Ciccia, Vasiliky Liakouli, Francesco Carubbi, Paola Di Benedetto, Piero Ruscitti, Onorina Berardicurti, Roberto Giacomelli, Paola Cipriani

Risultato della ricerca: Articlepeer review

20 Citazioni (Scopus)

Abstract

Introduction: Macrophage activation syndrome (MAS) is a severe, hyperinflammatory life-threatening syndrome, generally complicating different rheumatic diseases. Despite the severity of the disease, little is known about the pathogenic mechanisms and, thus, possible targeted therapies in the management of these patients. Areas covered: In this review, we aimed to update the current pathogenic knowledge of MAS, during rheumatic diseases, focusing mainly on immunologic abnormalities and on new possible therapeutic strategies. Expert commentary: The difficult pathogenic scenario of MAS, in which genetic defects, predisposing diseases, and triggers are mixed together with the high mortality rate, make it difficult to manage these patients. Although most efforts have been focused on investigating the disease in children, in recent years, several studies are trying to elucidate the possible pathogenic mechanism in adult MAS patients. In this context, genetic and immunological studies might lead to advances in the knowledge of pathogenic mechanisms and possible new therapeutic targets. In the future, the results of ongoing clinical trials are awaited in order to improve the management and, thus, the survival of these patients.
Lingua originaleEnglish
pagine (da-a)1041-1047
Numero di pagine7
RivistaExpert Review of Clinical Immunology
Volume13
Stato di pubblicazionePublished - 2017

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology

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