Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment
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Pediatric Rheumatology (Section Editor, G Martini)
Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment Francesco Baldo, MD1,2 Gisella Beretta, MD1,2 Jessica Tibaldi, MD3 Roberta Caorsi, MD3 Francesca Minoia, MD1,* Address *,1 Reumatologia Pediatrica – UOC Pediatria a Media Intensità di Cure, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Email: [email protected] 2 Università degli Studi di Milano, Milan, Italy 3 IRCCS Istituto Giannina Galsini, Genoa, Italy
* Springer Nature Switzerland AG 2020
This article is part of the Topical Collection on Pediatric Rheumatology Keywords Macrophage activation syndrome I Hemophagocytic lymphohistiocytosis I Hemophagocytic syndromes I Systemic juvenile idiopathic arthritis I Still’s disease I Cytokine storm syndromes
Abstract Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis,
Pediatric Rheumatology (Section Editor, G Martini) pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH.
Introduction Macrophage activation syndrome (MAS) refers to a severe and potentially fatal condition, due to an excessive and uncontrolled hyperinflammatory reaction, which might complicate rheumatologic diseases, mainly systemic juvenile idiopathic arthritis (sJIA) and its adult counterpart adult-onset Still’s disease (AOSD). MAS belongs to the spectrum of hemophagocytic lymphohistiocytosis (HLH) and is currently classified among its secondary forms, as a sHLH occurring in the context of a rheumatic disorder [1•, 2•] (Table 1). MAS was firstly reported by Hadchouel and colleagues who in
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