The Experts below are selected from a list of 62331 Experts worldwide ranked by ideXlab platform
Angelo Ravelli - One of the best experts on this subject based on the ideXlab platform.
-
Macrophage Activation Syndrome in pediatrics.
Pediatric Allergy and Immunology, 2020Co-Authors: Alessandra Alongi, Roberta Naddei, Laura De Miglio, Valentina Natoli, Angelo RavelliAbstract: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.
-
Macrophage Activation Syndrome
Hematology oncology clinics of North America, 2015Co-Authors: Angelo Ravelli, Sergio Davì, Francesca Minoia, Alberto Martini, Randy Q. CronAbstract: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.
-
Macrophage Activation Syndrome
Indian Journal of Rheumatology, 2012Co-Authors: Bianca Lattanzi, Sergio Davì, Alberto Martini, Silvia Rosina, Nicoletta Solari, Stefano Lanni, Giulia Bracciolini, Angelo RavelliAbstract: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.
-
Macrophage Activation Syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?
Lupus, 2007Co-Authors: Alejandra Beatriz Pringe, Nicolino Ruperto, Alberto Martini, L Trail, Antonella Buoncompagni, Anna Loy, Luciana Breda, Angelo RavelliAbstract: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...
-
Chapter 4 Macrophage Activation Syndrome
Handbook of Systemic Autoimmune Diseases, 2007Co-Authors: Angelo Ravelli, Alberto MartiniAbstract:Publisher Summary This chapter provides an overview of the clinical features, epidemiology, nomenclature, pathogenesis, diagnosis, and management of macrophage Activation Syndrome (MAS). MAS is a life-threatening complication of systemic inflammatory disorders seen most commonly in systemic juvenile idiopathic arthritis and is caused by the Activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. The clinical presentation of MAS is generally acute and occasionally dramatic. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important. The chapter outlines the preliminary diagnostic guidelines for the Syndrome are proposed. Cyclosporine A is effective in patients with corticosteroid resistant MAS, however, it is still unclear whether biologic agents have a role in the treatment of MAS.
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, 2019Co-Authors: Alexei A. Grom, Edward M. BehrensAbstract: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.
-
Macrophage Activation Syndrome: advances towards understanding pathogenesis
Current opinion in rheumatology, 2010Co-Authors: Alexei A. Grom, Elizabeth D. MellinsAbstract: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.
-
Macrophage Activation Syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?
Current opinion in rheumatology, 2003Co-Authors: Alexei A. GromAbstract: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.
-
Etanercept in the treatment of macrophage Activation Syndrome.
The Journal of rheumatology, 2001Co-Authors: Sampath Prahalad, Kevin E. Bove, David Dickens, Daniel J. Lovell, Alexei A. GromAbstract:Macrophage Activation Syndrome (MAS), a recognized complication of systemic juvenile rheumatoid arthritis (sJRA), has been associated with significant morbidity and mortality. Dysregulation of macrophage-lymphocyte interactions leading to uncontrolled proliferation of highly activated macrophages and massive release of proinflammatory cytokines including tumor necrosis factor-alpha (TNF-alpha) appears to be central to the pathogenesis of this Syndrome. Until now the mainstay of therapy has been corticosteroids and cyclosporin A. We describe a patient with MAS and sJRA successfully treated with the anti-TNF agent etanercept. The outcome in this patient suggests etanercept might be an effective therapeutic agent in MAS.
Alberto Martini - One of the best experts on this subject based on the ideXlab platform.
-
Macrophage Activation Syndrome
Hematology oncology clinics of North America, 2015Co-Authors: Angelo Ravelli, Sergio Davì, Francesca Minoia, Alberto Martini, Randy Q. CronAbstract: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.
-
Macrophage Activation Syndrome
Indian Journal of Rheumatology, 2012Co-Authors: Bianca Lattanzi, Sergio Davì, Alberto Martini, Silvia Rosina, Nicoletta Solari, Stefano Lanni, Giulia Bracciolini, Angelo RavelliAbstract: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.
-
Macrophage Activation Syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?
Lupus, 2007Co-Authors: Alejandra Beatriz Pringe, Nicolino Ruperto, Alberto Martini, L Trail, Antonella Buoncompagni, Anna Loy, Luciana Breda, Angelo RavelliAbstract: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...
-
Chapter 4 Macrophage Activation Syndrome
Handbook of Systemic Autoimmune Diseases, 2007Co-Authors: Angelo Ravelli, Alberto MartiniAbstract:Publisher Summary This chapter provides an overview of the clinical features, epidemiology, nomenclature, pathogenesis, diagnosis, and management of macrophage Activation Syndrome (MAS). MAS is a life-threatening complication of systemic inflammatory disorders seen most commonly in systemic juvenile idiopathic arthritis and is caused by the Activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. The clinical presentation of MAS is generally acute and occasionally dramatic. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important. The chapter outlines the preliminary diagnostic guidelines for the Syndrome are proposed. Cyclosporine A is effective in patients with corticosteroid resistant MAS, however, it is still unclear whether biologic agents have a role in the treatment of MAS.
-
macrophage Activation Syndrome in systemic juvenile rheumatoid arthritis successfully treated with cyclosporine
The Journal of Pediatrics, 1996Co-Authors: Angelo Ravelli, Fabrizio De Benedetti, S Viola, Alberto MartiniAbstract:A macrophage Activation Syndrome, possibly related to methotrexate toxicity, developed in a boy with systemic juvenile rheumatoid arthritis. Corticosteroid administration was ineffective, whereas a prompt response to cyclosporine was observed. Two months later, Pneumocystis carinii pneumonia developed.
Sonia Mínguez - One of the best experts on this subject based on the ideXlab platform.
-
Visceral leishmaniasis and macrophagic Activation Syndrome in a patient with rheumatoid arthritis under treatment with adalimumab
Joint bone spine, 2010Co-Authors: Anna Molto, Lourdes Mateo, Natalia Lloveras, Alejandro Olivé, Sonia MínguezAbstract:Abstract Background Visceral leishmaniasis is a protozoan infection usually asymptomatic, but can progress to fatal disease in immunocompromised hosts, especially in HIV patients. Visceral leishmaniasis is rare among patients under immunosuppressive therapies, and even more among patients under anti-TNF-α treatment, where only four cases have been described. Objective 1) To describe a patient with rheumatoid arthritis receiving adalimumab who developed fever, pancytopenia, splenomegaly, and extreme hyperferritinemia. 2) To perform a review of the published cases of visceral leishmaniasis and anti-TNF-α therapy, and cases of coexisting leishmaniasis and macrophagic Activation Syndrome by search in PubMed (period 1991–2008). Results Visceral leishmaniasis was established by bone marrow aspiration, and although there was no histological confirmation, according to HLH-2004 criteria, a secondary macrophagic Activation Syndrome was established. The patient had a favourable outcome. Conclusion We report herein the fifth case of visceral leishmaniasis in a patient under TNF-α therapy, and the first one, to our knowledge, presenting a consequent secondary macrophagic Activation Syndrome.
Knut Brockow - One of the best experts on this subject based on the ideXlab platform.
-
clonal mast cell Activation Syndrome with anaphylaxis to sulfites
International Archives of Allergy and Immunology, 2013Co-Authors: Liliana Cifuentes, Johannes Ring, Knut BrockowAbstract:Sulfites are rarely suspected as causative agents of immediate-type hypersensitivity. We report on a 49-year-old male patient who developed recurrent severe hypotension after food ingestion. A diagnosis of monoclonal mast cell Activation Syndrome was established. In the double-blind, placebo-controlled food challenge, the patient reacted to potassium metabisulfite with anaphylaxis.
-
Mast cell Activation Syndrome
Der Hautarzt; Zeitschrift fur Dermatologie Venerologie und verwandte Gebiete, 2013Co-Authors: Knut BrockowAbstract:BACKGROUND The description of a monoclonal mast cell Activation Syndrome in patients with anaphylaxis, who fulfill one or two minor-criteria of mastocytosis, has led to a search for new unrecognized mast cell Activation Syndromes. OBJECTIVE New classification of mast cell diseases including well-known diseases is provided in order to be able to better recognize and describe new entities. METHODS The term mast cell Activation has been defined by verifiable scientific objective and subjective criteria, and known and idiopathic mast cell Activation Syndromes have been classified. RESULTS Mast cell Activation cannot be defined by symptoms alone, as different diseases and conditions, including those with contribution of different cell types and somatization disorders may lead to similar symptoms. For this reason the preclinical checkpoint mast cell Activation was defined to require typical symptoms in combination with demonstration of mast cell mediator release in (an acute) episode(s) as well as with a good response to mast cell mediator-directed therapy. Mast cell Activation Syndromes were classified in primary (e.g. mastocytosis), secondary (e.g. IgE-mediated allergy) and idiopathic forms. CONCLUSION Only through a deeper understanding of mast cell diseases, can new previously unrecognized idiopathic mast cell Activation Syndrome entities be described and analyzed.
