Macrophage activation syndrome (MAS), also known as secondary haemophagocytic lymphohistocytosis (sHLH), is the most dangerous, potentially life-threatening complication in children with a variety of inflammatory disorders. Most often MAS is associated with systemic juvenile idiopathic arthritis (sJIA). Other rheumatic diseases, monogenic autoinflammatory disorders, infections, and malignancies may also be complicated by MAS. Cytokine storm in patients with severe COVID-19, post-COVID conditions, especially with multisystem inflammatory syndrome in children (MIS-C), again drew attention to MAS. However, there is a scarcity of data on MAS in children with SARS-CoV-2 infection and MIS-C. Frequently, it is very difficult to distinguish MAS from other complications. The innate immune system plays a central role in the initiation of MAS. Genetic predisposition and possible triggers on the background of active inflammation cause an increase in macrophage and T-lymphocyte activity, leading to cytokine storm that can result in tissue damage, multi-organ dysfunction, and even death. There is variability of treatment practice and outcomes of MAS in different countries depending on the availability of novel cytokine-targeted drugs.
The aim of this Research Topic is to present studies that define the pathophysiology, clinical phenotypes, and outcomes of macrophage activation syndrome in children with different inflammatory disorders, including SARS-CoV-2 infection. Special focus areas include sJIA, juvenile SLE, autoinflammatory diseases, MIS-C, Kawasaki disease.
Authors and researchers are invited to submit original research and review articles in the following areas: epidemiology, pathophysiology, clinical and laboratory features, treatment, and outcomes of MAS, and various inflammation diseases in children. Work on sJIA, MIS-C, autoinflammatory diseases, and inborn errors of immunity would be very welcome. We look forward to receiving single-centre and multi-centric studies defining and redefining the profile of MAS in the pediatric population.
Macrophage activation syndrome (MAS), also known as secondary haemophagocytic lymphohistocytosis (sHLH), is the most dangerous, potentially life-threatening complication in children with a variety of inflammatory disorders. Most often MAS is associated with systemic juvenile idiopathic arthritis (sJIA). Other rheumatic diseases, monogenic autoinflammatory disorders, infections, and malignancies may also be complicated by MAS. Cytokine storm in patients with severe COVID-19, post-COVID conditions, especially with multisystem inflammatory syndrome in children (MIS-C), again drew attention to MAS. However, there is a scarcity of data on MAS in children with SARS-CoV-2 infection and MIS-C. Frequently, it is very difficult to distinguish MAS from other complications. The innate immune system plays a central role in the initiation of MAS. Genetic predisposition and possible triggers on the background of active inflammation cause an increase in macrophage and T-lymphocyte activity, leading to cytokine storm that can result in tissue damage, multi-organ dysfunction, and even death. There is variability of treatment practice and outcomes of MAS in different countries depending on the availability of novel cytokine-targeted drugs.
The aim of this Research Topic is to present studies that define the pathophysiology, clinical phenotypes, and outcomes of macrophage activation syndrome in children with different inflammatory disorders, including SARS-CoV-2 infection. Special focus areas include sJIA, juvenile SLE, autoinflammatory diseases, MIS-C, Kawasaki disease.
Authors and researchers are invited to submit original research and review articles in the following areas: epidemiology, pathophysiology, clinical and laboratory features, treatment, and outcomes of MAS, and various inflammation diseases in children. Work on sJIA, MIS-C, autoinflammatory diseases, and inborn errors of immunity would be very welcome. We look forward to receiving single-centre and multi-centric studies defining and redefining the profile of MAS in the pediatric population.