TY - JOUR
T1 - Advances in immunopathogenesis of macrophage activation syndrome during rheumatic inflammatory diseases: toward new therapeutic targets?
AU - Ciccia, Francesco
AU - Triolo, Giovanni
AU - Guggino, Giuliana
AU - Liakouli, Vasiliky
AU - Carubbi, Francesco
AU - Di Benedetto, Paola
AU - Ruscitti, Piero
AU - Berardicurti, Onorina
AU - Giacomelli, Roberto
AU - Cipriani, Paola
PY - 2017
Y1 - 2017
N2 - 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.
AB - 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.
KW - Immunology
KW - Immunology and Allergy
KW - Macrophage activation syndrome
KW - adult onset Stillâs disease
KW - cytokine
KW - hyperferritinemic syndrome
KW - inflammation
KW - macrophage
KW - systemic juvenile idiopathic arthritis
KW - Immunology
KW - Immunology and Allergy
KW - Macrophage activation syndrome
KW - adult onset Stillâs disease
KW - cytokine
KW - hyperferritinemic syndrome
KW - inflammation
KW - macrophage
KW - systemic juvenile idiopathic arthritis
UR - http://hdl.handle.net/10447/245541
M3 - Article
VL - 13
SP - 1041
EP - 1047
JO - Expert Review of Clinical Immunology
JF - Expert Review of Clinical Immunology
SN - 1744-666X
ER -