• There is uncontrolled activation and proliferation of macrophages, and T lymphocytes, with a marked increase in circulating cytokines, such as IFN-gamma, and GM-CSF. (wikipedia.org)
  • Macrophage activation syndrome (MAS) is a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T lymphocytes and macrophages. (nih.gov)
  • Synonymous with secondary hemophagocytic lymphohistiocytosis, macrophage activation syndrome (MAS) is a term used by rheumatologists to describe a potentially life-threatening complication of systemic inflammatory disorders, most commonly systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE). (nih.gov)
  • Background: Multisystem inflammatory syndrome in children (MIS-C) is a potentially life-threatening hyperinflammatory syndrome that occurs after primary SARS-CoV-2 infection. (ssrn.com)
  • The pathogenesis of MIS-C remains undefined, and whether specific inflammatory biomarker patterns can distinguish MIS-C from other hyperinflammatory syndromes including Kawasaki disease (KD) and macrophage activation syndrome (MAS) is unknown. (ssrn.com)
  • However, M1 and non-activated (M0) macrophages, but not M2 macrophages, significantly up-regulate inflammatory factors upon viral infection. (nature.com)
  • Recent studies 5 , 6 on the immunity of COVID-19 patients indicated that the cells damaged by SARS-CoV-2 infection triggered innate inflammation in the lungs, which is largely mediated by pro-inflammatory macrophages and granulocytes. (nature.com)
  • In addition to local damage, the pro-inflammatory macrophages release cytokines/chemoattractants and prime adaptive immune cell responses In some cases, this lead to cytokine release syndromes including macrophage activation syndrome (MAS) 7 , followed by respiratory and even multi-organ failure 5 . (nature.com)
  • The activation of macrophages confers a heterogeneous function and plasticity depending on the microenvironment and duration of stimulation, which include a spectrum of phenotypes associated with different inflammatory responses. (nature.com)
  • American College of Rheumatology Clinical Guidance for Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 and Hyperinflammation in Pediatric COVID-19: Version 2. (nih.gov)
  • The elevated levels of inflammatory cytokines and chemokines (e.g., interleukin [IL] 6, IL-8, macrophage inflammatory protein 1α and 1β, monocyte chemoattractant protein 1, and macrophage colony-stimulating factor) and immunomodulatory cytokines (e.g. (cdc.gov)
  • We have developed models of cytokine shock syndromes to identify the critical cell death and inflammatory pathways involved, and are using biochemical experiments and genetic manipulation (e.g. cell type-specific targeting) in order to define new therapeutic targets. (edu.au)
  • Enigma of COVID-19: is "multisystem inflammatory syndrome in adults" (MIS-A) predictable? (biomedcentral.com)
  • Severe inflammation and one or more extrapulmonary organ dysfunctions have been reported and this clinical picture is defined as "multisystem inflammatory syndrome in adults" (MIS-A) in severe coronavirus disease-2019 (COVID-19). (biomedcentral.com)
  • Eight study participants with high levels of an inflammatory cytokine known as IL-18, clubbing of the fingernails and lung disease and a potentially fatal inflammatory complication known as macrophage activation syndrome (MAS)-but no disease-causing genetic mutation-had a disease dubbed IL-18PAP-MAS. (nih.gov)
  • MAS stands for Macrophage Activation Syndrome, and it is an inflammatory response stemming from an over-reactive immune response, similar to the "cytokine storm" that's being widely discussed (which vitamin C helps prevent, according to published research). (naturalnews.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages secreting high amounts of inflammatory cytokines. (biomedcentral.com)
  • BACKGROUND Macrophage activation syndrome (MAS) is a life-threatening complication of Still's disease (SD) characterized by overt immune cell activation and cytokine storm. (jci.org)
  • The severity of systemic inflammation in response to human coronavirus family members has features reminiscent of a cytokine storm or macrophage activation syndrome (MAS), also known as secondary haemophagocytic lymphohistocytosis (sHLH). (naturalnews.com)
  • Cytokine storm syndrome is a diverse set of conditions that can result in a cytokine storm. (wikipedia.org)
  • Cytokine storm syndromes include familial hemophagocytic lymphohistiocytosis , Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, systemic or non-systemic juvenile idiopathic arthritis -associated macrophage activation syndrome , NLRC4 macrophage activation syndrome, cytokine release syndrome and sepsis . (wikipedia.org)
  • The term "cytokine storm" is often loosely used interchangeably with cytokine release syndrome (CRS) but is more precisely a differentiable syndrome that may represent a severe episode of cytokine release syndrome or a component of another disease entity, such as macrophage activation syndrome . (wikipedia.org)
  • [19] Cytokine storm has also been implicated in hantavirus pulmonary syndrome . (wikipedia.org)
  • Mazodier K, Marin V, Novick D, Farnarier C, Robitail S, Schleinitz N. Severe imbalance of IL-18/IL-18BP in patients with secondary hemophagocytic syndrome. (medscape.com)
  • The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to the proposal to rename it according to the contemporary classification of histiocytic disorders. (nih.gov)
  • 26. Exploring Macrophage Activation Syndrome Secondary to Systemic Lupus Erythematosus in Adults: A Systematic Review of the Literature. (nih.gov)
  • The hemophagocytic syndromes may be classified as either primary or secondary. (the-hospitalist.org)
  • In contrast, secondary HPS is characterized by the polyclonal accumulation of activated macrophages in patients with underlying infectious, malignant, or rheumatologic diseases. (the-hospitalist.org)
  • Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis /multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment? (acibadem.com.tr)
  • Heme application did not only affect macrophage function in vivo upon secondary stimulation. (egis-online.eu)
  • Cytokine shock syndromes are frequently lethal conditions often triggered by infectious pathogens in genetically susceptible people (e.g. sepsis, hemophagocytic lymphohistiocytosis, macrophage activation syndrome). (edu.au)
  • Here, we report two cases of disseminated toxoplasmosis in critically ill adolescents after HCT presenting with hyperferritinemic sepsis and multiple organ dysfunction syndrome (MODS). (frontiersin.org)
  • Macrophage activation syndrome is a severe, potentially life-threatening, complication of several chronic rheumatic diseases of childhood. (wikipedia.org)
  • Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases (i.e SLE, Juvenile idiopathic arthritis, Adult onset Still's disease). (medicinespecifics.com)
  • Macrophage activation syndrome (MAS) is a life-threatening complication that occurs in 10-19% of patients with AOSD (hereafter referred to as AOSD-MAS), with a short-term mortality rate as high as 20% [2] . (researchsquare.com)
  • A variant (polymorphism) of the UNC13D gene has been associated with a higher risk of a complication called macrophage activation syndrome in people with systemic juvenile idiopathic arthritis (SJIA). (nih.gov)
  • Macrophage activation syndrome is a life-threatening complication of SJIA with symptoms similar to those of familial hemophagocytic lymphohistiocytosis, including fever, an enlarged liver and spleen, liver damage, and low numbers of blood cells. (nih.gov)
  • They stumbled upon a particularly fatal mix of infections, which resembled a complication in humans known as either sHLH or macrophage activation syndrome. (yale.edu)
  • Mutations in the gene NLRC4 have been associated with a potentially life-threatening autoinflammatory disorder called macrophage activation syndrome (MAS). (nih.gov)
  • Cytosolic inflammasome protein complexes drive cell death and inflammation, and while they are key for innate immunity against pathogen infections, their excess activation causes a variety of autoinflammatory conditions. (edu.au)
  • They were also more likely to experience symptoms similar to those of known autoinflammatory, interferon-mediated diseases, such as SAVI , CANDLE and Aicardi-Goutières syndrome . (nih.gov)
  • In several cases, autoinflammatory diseases are associated with increased activation of some cellular molecules known as JAKs and STATs. (nih.gov)
  • C3101 Genetic Disorder C26323 Hematological Disorder C118468 Pediatric Rheumatology Terminology C116794 Blau Syndrome Blau Syndrome Pediatric Granulomatous Arthritis An autoinflammatory disease caused by a NOD2 gene mutation, usually presenting in children younger than age four, and characterized by granulomatous dermatitis, arthritis with synovitis, and uveitis. (nih.gov)
  • Cryopyrinopathy A group of autoinflammatory syndromes caused by mutations in the NLRP3 gene. (nih.gov)
  • A family of autoinflammatory diseases caused by gain of function mutations in NLRP3, which encodes cryopyrin, resulting in inflammasome activation and enhanced IL-1 beta and IL-18 production. (nih.gov)
  • C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C119053 Familial Cold Autoinflammatory Syndrome Familial Cold Autoinflammatory Syndrome FCAS An autoinflammatory disease caused by mutations in the NLRP3 gene which encodes cryopyrin. (nih.gov)
  • [4] The viruses can invade lung epithelial cells and alveolar macrophages to produce viral nucleic acid, which stimulates the infected cells to release cytokines and chemokines, activating macrophages, dendritic cells, and others. (wikipedia.org)
  • 21. Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review. (nih.gov)
  • In addition, we previously demonstrated that accumulated macrophages synthesized ferritin in the lung and bone marrow of a patient with RP-ILD associated with DM [ 9 ]. (hindawi.com)
  • The COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), results in life-threatening disease in a minority of patients, especially elderly people and those with co-morbidities such as obesity and diabetes. (nature.com)
  • Severe disease is characterized by dysregulated cytokine release, pneumonia and acute lung injury, which can rapidly progress to acute respiratory distress syndrome, disseminated intravascular coagulation, multisystem failure and death. (nature.com)
  • We further discuss potential mechanisms of inflammasome activation and clinical efforts currently under way to suppress inflammation to prevent or ameliorate severe COVID-19. (nature.com)
  • Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus responsible for COVID-19, has so far infected more than 190 million people and caused death of more than 4.1 million people worldwide. (nature.com)
  • As many as 10-15% of patients develop severe pneumonia, with some cases progressing to hypoxia and acute respiratory distress syndrome (ARDS), which requires mechanical ventilation in a critical care setting and has high mortality. (nature.com)
  • Nonetheless, several studies are now accumulating that support direct (infection-induced) and indirect inflammasome activation and the critical role of inflammasomes in severe COVID-19. (nature.com)
  • The infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has already caused more than 500 million Coronavirus Disease-2019 (COVID-19) cases internationally ( https://google.org/crisisresponse/covid19-map ). (nature.com)
  • An evaluation of 2 cohorts of patients with EVD revealed that a marker of macrophage activation (sCD163) but not T-cell activation (sCD25) was associated with severe and fatal EVD. (cdc.gov)
  • Hypercytokinemia accompanied by severe clinical disease seen in EVD is reminiscent of what has been described for macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH). (cdc.gov)
  • Coronavirus disease-2019 (COVID-19) is a disease caused by severe acute respiratory syndrome coronavirus-2 (SARS CoV-2), first detected in Wuhan, China in December 2019, and then affected the whole world and caused a pandemic [ 1 ]. (biomedcentral.com)
  • Treat severe or life-threatening CRS with tocilizumab or tocilizumab and corticosteroids .Immune Effector Cell-Associated Neurotoxicity Syndrome (ICANS), which may be fatal or life-threatening, occurred following treatment with ciltacabtagene autoleucel, including before CRS onset, concurrently with CRS, after CRS resolution, or in the absence of CRS. (drugs.com)
  • However, the specific links between air pollution and severe acute respiratory syndrome-coronavirus-2 infection remain unclear. (ersjournals.com)
  • In late 2019, the city of Wuhan, China became the epicenter of a pneumonia epidemic due to a new coronavirus, severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2), which causes a disease given the name coronavirus disease 2019 (COVID-19) in February 2020 by the World Health Organization [ 1 ]. (ersjournals.com)
  • Multiple organ failure and cardiopulmonary complications, such as myopericarditis, pulmonary embolism and acute respiratory distress syndrome, represent some of the major complications of severe COVID-19. (ersjournals.com)
  • KYMRIAH may cause side effects that are severe or life-threatening, such as cytokine release syndrome (CRS) and neurological toxicities. (kymriah.com)
  • Bone marrow examination reveals numerous well differentiated macrophages actively phagocytosing hematopoietic elements. (nih.gov)
  • Immunologically, SARS-CoV-2 infects cells that express the angiotensin-converting enzyme 2 receptor, including cells of the respiratory tract, endothelial cells, and likely hematopoietic cells, including macrophages. (ccjm.org)
  • Impact of cytoreduction and remission status on hematopoietic cell transplantation outcomes in pediatric myelodysplastic syndrome and related disorders. (dana-farber.org)
  • Our studies indicate differential immune response patterns in distinct macrophage phenotypes, which could lead to a range of COVID-19 disease severity. (nature.com)
  • A genetic mechanism shared by different phenotypes of cryopyrin-associated periodic syndromes. (congressmed.com)
  • A rare hemophagocytic syndrome characterized by excessive activation and proliferation of macrophages and T cells occurring in the context of a variety of diseases, including infections, neoplasms, rheumatic disorders, and leading to sudden onset of persistent fever, lymphadenopathy, and hepatosplenomegaly. (nih.gov)
  • Risk of neurologic toxicities including immune effector cell-associated neurotoxicity syndrome, parkinsonism, Guillain-Barré syndrome, peripheral neuropathies, cranial nerve palsies. (renalandurologynews.com)
  • 25. Recurrent macrophage activation syndrome as the initial manifestation of paediatric systemic lupus erythematosus. (nih.gov)
  • Warning: Cytokine release syndrome, neurological toxicities, HLH/MAS and prolonged and recurrent cytopenia Cytokine Release Syndrome (CRS), including fatal or life-threatening reactions, occurred in patients following treatment with ciltacabtagene autoleucel. (drugs.com)
  • He has a special interest in pathologic inflammation and immune dysregulation, dealing with diseases like macrophage activation syndrome. (choa.org)
  • Macrophage activation in these disorders may be attributed to dysregulation of cytokines such as IL-1, IL-6, IFN-y and TNF-a. (the-hospitalist.org)
  • Both patients developed hyperferritinemia with multiple organ dysfunction syndrome and profound immune dysregulation, which progressed to death despite maximal medical therapies. (frontiersin.org)
  • 27. Acute pancreatitis and macrophage activation syndrome in pediatric systemic lupus erythematosus: case-based review. (nih.gov)
  • 29. Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. (nih.gov)
  • In this study, we modified the classical regimen of the hemophagocytic lymphohistiocytosis-04 protocol and evaluated the efficacy and safety of short-term, low-dose etoposide in patients with refractory macrophage activation syndrome (MAS) associated with adult-onset Still's disease (AOSD). (researchsquare.com)
  • COVID-19 associated hyperinflammation overlaps with macrophage activation syndrome (MAS) in several ways in some patients. (biomedcentral.com)
  • 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. (medscape.com)
  • Grom AA, Passo M. Macrophage activation syndrome in systemic juvenile rheumatoid arthritis. (medscape.com)
  • Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13-4 polymorphisms. (medscape.com)
  • Systemic juvenile idiopathic arthritis and macrophage activation syndrome: update on pathogenesis and treatment. (medscape.com)
  • Maeno N, Takei S, Imanaka H, Yamamoto K, Kuriwaki K, Kawano Y. Increased interleukin-18 expression in bone marrow of a patient with systemic juvenile idiopathic arthritis and unrecognized macrophage-activation syndrome. (medscape.com)
  • 22. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. (nih.gov)
  • Because macrophage activation syndrome (MAS) is a serious condition that can follow a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are critical. (medscape.com)
  • Dengue infection associated hemophagocytic syndrome: Therapeutic interventions and outcome. (cdc.gov)
  • It is urgent to understand the interactions among permissive cells, macrophages, and the SARS-CoV-2 virus, thereby offering important insights into effective therapeutic strategies. (nature.com)
  • Grom AA, Villanueva J, Lee S, Goldmuntz EA, Passo MH, Filipovich A. Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome. (medscape.com)
  • Crimean-Congo hemorrhagic fever: five patients with hemophagocytic syndrome. (cdc.gov)
  • Cytokine Release Syndrome (CRS), including life-threatening reactions, occurred in patients receiving TECARTUS. (nih.gov)
  • 30. Reactive hemophagocytic syndrome in adult Korean patients with systemic lupus erythematosus: a case-control study and literature review. (nih.gov)
  • 34. [Clinical characteristics and treatment outcomes of macrophage activation syndrome in adults: A case series of 67 patients]. (nih.gov)
  • Therefore, we conducted a series of experiments to investigate the immune response of M1φ, M2φ, or non-activated macrophages (M0φ) to SARS-CoV-2 infection, which could reflect the differential responses seen in patients at different stages of macrophage activation. (nature.com)
  • Provide supportive care and/or corticosteroids as needed.Parkinsonism and Guillain-Barré syndrome and their associated complications resulting in fatal or life-threatening reactions have occurred following treatment with ciltacabtagene autoleuce.Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS), including fatal and life-threatening reactions, occurred in patients following treatment with ciltacabtagene autoleucel. (drugs.com)
  • 38. Unmasking of systemic lupus erythematosus in a patient with hemophagocytic lymphohistiocytosis- macrophage activation syndrome (HLA-MAS) and diffuse alveolar hemorrhage. (nih.gov)
  • These data suggest that host macrophage activation contributes to EVD pathogenesis and that directed antiinflammatory therapies could be beneficial in the treatment of EVD. (cdc.gov)
  • The bone marrow aspirate shown demonstrates macrophage hemophagocytosis of non-nucleated red blood cells, consistent with hemophagocytic syndrome (HPS). (the-hospitalist.org)
  • 0.75) for HPS in the appropriate clinical setting, though histologic demonstration of hemophagocytosis (ingestion of red blood cells by cytokine-activated macrophages) in the bone marrow is diagnostic. (the-hospitalist.org)
  • When Do Symptoms of Macrophage activation syndrome Begin? (nih.gov)
  • Natural killer cell dysfunction: A common pathway in systemic-onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis? (medscape.com)
  • Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A. Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome. (medscape.com)
  • 35. Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis. (nih.gov)
  • The mutations cause aggregation and continual activation of a cellular sensor (shown in red in the image) that triggers inflammation. (nih.gov)
  • Dysfunctional immune responses contribute critically to the progression of Coronavirus Disease-2019 (COVID-19), with macrophages as one of the main cell types involved. (nature.com)
  • The UNC13D gene variant associated with macrophage activation syndrome in people with SJIA consists of a specific combination of individual changes in 12 DNA building blocks (nucleotides). (nih.gov)
  • One paper provides updates on the pharmacologic management of JIA, focusing on treatment of oligoarthritis, temporomandibular (TMJ) arthritis and systemic JIA (sJIA), with and without macrophage activation syndrome (MAS). (eurekalert.org)
  • Cytokine release via activation of JAK/STAT signalling pathway following SARS-Cov-2 infection resulting in ARDS related to COVID-19. (wikipedia.org)
  • This type of hyperinflammatory state is reminiscent of 2 rheumatologic disorders known as macrophage activation syndrome and hemophagocytic lymphohistiocytosis, which are characterized by macrophage and T-cell activation. (cdc.gov)
  • Interleukin-18 diagnostically distinguishes and pathogenically promotes human and murine macrophage activation syndrome. (medscape.com)
  • 36. Macrophage activation syndrome triggered by active systemic lupus erythematosus : Successful treatment by interleukin-1 inhibition (anakinra). (nih.gov)
  • Immune Effector Cell-Associated Neurotoxicity Syndrome (ICANS), which may be fatal or life-threatening, occurred following treatment with CARVYKTI ® , including before CRS onset, concurrently with CRS, after CRS resolution, or in the absence of CRS. (financialcontent.com)
  • The discovery of a fatal mix of infections in mice will allow scientists to study macrophage activation syndrome in an animal model for the first time. (yale.edu)
  • Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities? (nih.gov)
  • Over a 10-day span in mid-April, Riphagen et al observed a cluster of 8 previously healthy children (5 boys, 3 girls, age 4 - 14 years) with hyperinflammatory shock with features of Kawasaki disease (KD), atypical KD, or toxic shock syndrome (TSS). (aad.org)
  • Macrophage activation syndrome in rheumatic diseases in children]. (medscape.com)
  • However, continual activation of this process can lead to serious diseases like MAS. (nih.gov)
  • 28. Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature. (nih.gov)
  • 32. Systemic lupus erythematosus associated with development of macrophage activation syndrome and disseminated aspergillosis. (nih.gov)
  • With this model, the research team learned that in animals with sHLH, specialized cells known as macrophages are over-stimulated and start devouring immune cells and red blood cells. (yale.edu)
  • Primary HPS is an autosomal recessive disorder most commonly seen in children and characterized by the polyclonal accumulation of T-lymphocytes and activated macrophages. (the-hospitalist.org)
  • Here, we establish a lung and macrophage co-culture system derived from human pluripotent stem cells (hPSCs), modeling the host-pathogen interaction in SARS-CoV-2 infection. (nature.com)
  • Background/Purpose: Macrophage activation syndrome (MAS) constitutes over 5% of multi-organ dysfunction syndrome in adults, leading to 60-70% mortality without early treatment. (acrabstracts.org)
  • Billiau AD, Roskams T, Van Damme-Lombaerts R, Matthys P, Wouters C. Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alpha-producing macrophages. (medscape.com)
  • van der Ven AJ , Netea MG , van der Meer JW , de Mast Q . Ebola virus disease has features of hemophagocytic lymphohistiocytosis syndrome. (cdc.gov)
  • 37. Clinical analysis of macrophage activation syndrome in adult rheumatic disease: A multicenter retrospective study. (nih.gov)