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Angelo Ravelli – One of the best experts on this subject based on the ideXlab platform.

  • Macrophage Activation Syndrome in pediatrics.
    Pediatric Allergy and Immunology, 2020
    Co-Authors: Alessandra Alongi, Roberta Naddei, Laura De Miglio, Valentina Natoli, Angelo Ravelli

    Abstract:

    Macrophage Activation Syndrome (MAS) is a serious, potentially life-threatening, hyperinflammatory condition, which belongs to the spectrum of hemophagocytic lymphohistiocytosis (HLH) and can complicate several immunologic and rheumatic disorders. MAS is characterized by a dysfunctional immune response that is similar to that seen in other forms of HLH. Because MAS may pursue a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are fundamental. Recently, a set of classification criteria for MAS complicating sJIA has been developed through a multinational collaborative effort. High-dose parenteral corticosteroids remain the mainstay of treatment of MAS.

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  • Macrophage Activation Syndrome
    Hematology oncology clinics of North America, 2015
    Co-Authors: Angelo Ravelli, Sergio Davì, Francesca Minoia, Alberto Martini, Randy Q. Cron

    Abstract:

    Macrophage Activation Syndrome (MAS) is a potentially life-threatening complication of rheumatic disorders that occurs most commonly in systemic juvenile idiopathic arthritis. In recent years, there have been several advances in the understanding of the pathophysiology of MAS. Furthermore, new classification criteria have been developed. Although the place of cytokine blockers in the management of MAS is still unclear, interleukin-1 inhibitors represent a promising adjunctive therapy, particularly in refractory cases.

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  • Macrophage Activation Syndrome
    Indian Journal of Rheumatology, 2012
    Co-Authors: Bianca Lattanzi, Sergio Davì, Alberto Martini, Silvia Rosina, Nicoletta Solari, Stefano Lanni, Giulia Bracciolini, Angelo Ravelli

    Abstract:

    Abstract Macrophage Activation Syndrome (MAS) is a serious, potentially life-threatening complication of rheumatic disorders, which is seen most commonly in systemic juvenile idiopathic arthritis (sJIA). It is characterised clinically by unremitting high fever, pancytopaenia, hepatosplenomegaly, hepatic dysfunction, encephalopathy, coagulation abnormalities and sharply increased levels of ferritin. The pathognomonic feature of the Syndrome is seen on bone marrow examination, which frequently, though not always, reveals numerous morphologically benign macrophages exhibiting haemophagocytic activity. Macrophage Activation Syndrome is overt in 10% of children with sJIA but occurs subclinically in another 30–40%. Because MAS can follow a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are essential. However, it is difficult to distinguish sJIA disease flare, infectious complications or medication side effects from MAS. A multinational collaborative effort aimed to develop diagnostic criteria for MAS in sJIA is under way. Although, the pathogenesis of MAS is unclear, the hallmark of the Syndrome is an uncontrolled Activation and proliferation of T lymphocytes and macrophages, leading to massive hypersecretion of pro-inflammatory cytokines. Mutations in cytolytic pathway genes are increasingly being recognised in children who develop MAS as part of sJIA. Recently, a mouse model of MAS dependent on repeated stimulation through toll-like receptors was developed. The first-line therapy of MAS complicating sJIA is based on the parenteral administration of high doses of corticosteroids, with or without cyclosporine A. There is increasing evidence that biological therapies, particularly interleukin-1 inhibitors, represent a valuable adjunct to corticosteroids and cyclosporine A in treating MAS complicating sJIA.

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Alexei A. Grom – One of the best experts on this subject based on the ideXlab platform.

  • Macrophage Activation Syndrome in Rheumatic Diseases
    Textbook of Autoinflammation, 2019
    Co-Authors: Alexei A. Grom, Edward M. Behrens

    Abstract:

    Macrophage Activation Syndrome is a hemophagocytic Syndrome presenting as a complication of a rheumatic disease. Excessive Activation and expansion of T lymphocytes and macrophagic histiocytes in MAS leads to a cytokine storm and hyperinflammation associated with extreme hyperferritinemia, cytopenias, liver dysfunction and coagulopathy resembling disseminated intravascular coagulation. It is a life-threatening condition and may progress to multiple organ failure. High dose glucocorticoids and cyclosporine A are most commonly used to treat MAS. Anakinra and intravenous immunoglobulin may be effective in some patients. Etoposide should be considered in more severe cases. Treatments under investigation include strategies aimed at neutralization of IFN-γ and IL-18.

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  • Macrophage Activation Syndrome: advances towards understanding pathogenesis
    Current opinion in rheumatology, 2010
    Co-Authors: Alexei A. Grom, Elizabeth D. Mellins

    Abstract:

    Purpose of review
    Macrophage Activation Syndrome (MAS), a major cause of morbidity and mortality in pediatric rheumatology, is most strongly associated with systemic juvenile idiopathic arthritis (SJIA). There are no validated diagnostic criteria and early diagnosis is difficult. This review summarizes the progress in understanding of MAS pathophysiology that may help define specific diagnostic biomarkers.

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  • Macrophage Activation Syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?
    Current opinion in rheumatology, 2003
    Co-Authors: Alexei A. Grom

    Abstract:

    Purpose of the review One of the most perplexing features of systemic-onset juvenile rheumatoid arthritis is the association with macrophage Activation Syndrome, a life-threatening complication caused by excessive Activation and proliferation of T cells and macrophages. The main purpose of the review is to summarize current understanding of the relation between macrophage Activation Syndrome and other clinically similar hemophagocytic disorders. Recent findings Clinically, macrophage Activation Syndrome has strong similarities with familial and virus-associated reactive hemophagocytic lymphohistiocytosis. The better understood familial hemophagocytic lymphohistiocytosis is a constellation of rare, autosomal recessive immune disorders. The most consistent immunologic abnormalities in patients with familial hemophagocytic lymphohistiocytosis are decreased natural killer and cytotoxic cell functions. In approximately one third of familial hemophagocytic lymphohistiocytosis patients, these immunologic abnormalities are secondary to mutations in the gene encoding perforin, a protein that mediates cytotoxic activity of natural killer and cytotoxic CD8 + T cells. Several recent studies have suggested that profoundly depressed natural killer cell activity and abnormal levels of perforin expression may be a feature of macrophage Activation Syndrome in systemic-onset juvenile rheumatoid arthritis as well. Although it has been proposed that in both hemophagocytic lymphohistiocytosis and macrophage Activation Syndrome, natural killer and cytotoxic cell dysfunction may lead to inadequate control of cellular immune responses, the exact nature of such dysregulation and the relation between macrophage Activation Syndrome and hemophagocytic lymphohistiocytosis still remain to be determined.

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Alberto Martini – One of the best experts on this subject based on the ideXlab platform.

  • Macrophage Activation Syndrome
    Hematology oncology clinics of North America, 2015
    Co-Authors: Angelo Ravelli, Sergio Davì, Francesca Minoia, Alberto Martini, Randy Q. Cron

    Abstract:

    Macrophage Activation Syndrome (MAS) is a potentially life-threatening complication of rheumatic disorders that occurs most commonly in systemic juvenile idiopathic arthritis. In recent years, there have been several advances in the understanding of the pathophysiology of MAS. Furthermore, new classification criteria have been developed. Although the place of cytokine blockers in the management of MAS is still unclear, interleukin-1 inhibitors represent a promising adjunctive therapy, particularly in refractory cases.

    Free Register to Access Article

  • Macrophage Activation Syndrome
    Indian Journal of Rheumatology, 2012
    Co-Authors: Bianca Lattanzi, Sergio Davì, Alberto Martini, Silvia Rosina, Nicoletta Solari, Stefano Lanni, Giulia Bracciolini, Angelo Ravelli

    Abstract:

    Abstract Macrophage Activation Syndrome (MAS) is a serious, potentially life-threatening complication of rheumatic disorders, which is seen most commonly in systemic juvenile idiopathic arthritis (sJIA). It is characterised clinically by unremitting high fever, pancytopaenia, hepatosplenomegaly, hepatic dysfunction, encephalopathy, coagulation abnormalities and sharply increased levels of ferritin. The pathognomonic feature of the Syndrome is seen on bone marrow examination, which frequently, though not always, reveals numerous morphologically benign macrophages exhibiting haemophagocytic activity. Macrophage Activation Syndrome is overt in 10% of children with sJIA but occurs subclinically in another 30–40%. Because MAS can follow a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are essential. However, it is difficult to distinguish sJIA disease flare, infectious complications or medication side effects from MAS. A multinational collaborative effort aimed to develop diagnostic criteria for MAS in sJIA is under way. Although, the pathogenesis of MAS is unclear, the hallmark of the Syndrome is an uncontrolled Activation and proliferation of T lymphocytes and macrophages, leading to massive hypersecretion of pro-inflammatory cytokines. Mutations in cytolytic pathway genes are increasingly being recognised in children who develop MAS as part of sJIA. Recently, a mouse model of MAS dependent on repeated stimulation through toll-like receptors was developed. The first-line therapy of MAS complicating sJIA is based on the parenteral administration of high doses of corticosteroids, with or without cyclosporine A. There is increasing evidence that biological therapies, particularly interleukin-1 inhibitors, represent a valuable adjunct to corticosteroids and cyclosporine A in treating MAS complicating sJIA.

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  • Macrophage Activation Syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?
    Lupus, 2007
    Co-Authors: Alejandra Beatriz Pringe, Nicolino Ruperto, Alberto Martini, L Trail, Antonella Buoncompagni, Anna Loy, Luciana Breda, Angelo Ravelli

    Abstract:

    Macrophage Activation Syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the Activation and uncontrolled proliferation of T lymphocytes and macr…

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