Macrophage Activation Syndrome

A serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T-LYMPHOCYTES and MACROPHAGES. It is seen predominantly in children with systemic onset JUVENILE IDIOPATHIC ARTHRITIS.

Also Known As:

Networked: 150 relevant articles (14 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Juvenile Rheumatoid Arthritis (Juvenile Idiopathic Arthritis)
2.	Hemophagocytic Lymphohistiocytosis (Hemophagocytic Syndrome)
3.	Adult-Onset Still's Disease
4.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
5.	Arthritis (Polyarthritis)

Experts

1.	Grom, Alexei A: 7 articles (01/2016 - 09/2003)
2.	Martini, Alberto: 5 articles (01/2016 - 12/2012)
3.	Yokota, Shumpei: 5 articles (04/2015 - 01/2004)
4.	Yachie, Akihiro: 4 articles (10/2015 - 09/2010)
5.	Shimizu, Masaki: 4 articles (10/2015 - 09/2010)
6.	Wouters, Carine: 4 articles (08/2015 - 02/2005)
7.	Cron, Randy Q: 4 articles (01/2014 - 02/2011)
8.	Dinarello, Charles A: 4 articles (12/2013 - 11/2005)
9.	Brunner, Hermine I: 3 articles (01/2016 - 03/2007)
10.	Ilowite, Norman T: 3 articles (01/2016 - 03/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Macrophage Activation Syndrome:

1.	Cyclosporine (Ciclosporin)FDA LinkGeneric 07/01/1993 - "Cyclosporin A was effective in two patients and may be of value in the management of the macrophage-activation syndrome. " 01/01/2001 - "Dramatic efficacy of cyclosporine A in macrophage activation syndrome." 08/01/2011 - "This has important implications in rheumatology where cyclosporine is a life saving medication used in macrophage activation syndrome." 09/01/1997 - "Finally, a report added confirmation to the successful use of cyclosporine for macrophage activation syndrome in JRA." 12/01/2006 - "Macrophage activation syndrome (MAS) is one of the serious complications of juvenile rheumatoid arthritis (JRA) and recently, cyclosporine A has been found to be effective in patients with corticosteroid-resistant MAS. "
2.	Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link 05/01/2013 - "Higher-dose Anakinra is effective in a case of medically refractory macrophage activation syndrome." 11/01/2015 - "Refractory macrophage activation syndrome in a patient with SLE and APLA syndrome - Successful use of PET- CT and Anakinra in its diagnosis and treatment." 12/01/2012 - "Successful treatment of macrophage activation syndrome complicating adult Still disease with anakinra." 04/01/2010 - "Macrophage activation syndrome treated with anakinra." 11/01/2008 - "A case of macrophage activation syndrome successfully treated with anakinra."
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/2011 - "Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids." 10/01/2010 - "Septic shock was suspected, but he was diagnosed with macrophage activation syndrome and treated with corticosteroids and intravenous immunoglobulin and later discharged from the ICU." 01/01/2004 - "To avoid disease progression to macrophage activation syndrome and the adverse effects of high-dose corticosteroids, it might be reasonable to inhibit the formation of IL-6/IL-6R complex in order to block the binding to gp130 receptor, a biologically active receptor for IL-6. " 01/01/2002 - "These changes include the avoidance of high-dose rabbit antithymocyte serum in patients who received T-cell-depleted grafts, use of corticosteroids with granulocyte colony-stimulating factor during stem cell mobilization and as prophylaxis for the engraftment syndrome in MS, lung radiation shielding in SSc, and multiple precautions against the macrophage activation syndrome in JIA. " 01/01/2014 - "Additionally, interleukin-1 blockade appears to be effective treatment of macrophage activation syndrome, one of the most dangerous complications of JIA; specifically, anakinra in combination with cyclosporine and corticosteroids may obviate the need for cytotoxic approaches. "
4.	canakinumabFDA Link 01/01/2016 - "In pivotal trials, canakinumab has been shown to be effective in the treatment of systemic juvenile idiopathic arthritis (JIA), but reported adverse events have included macrophage activation syndrome (MAS). " 01/01/2016 - "Rate and Clinical Presentation of Macrophage Activation Syndrome in Patients With Systemic Juvenile Idiopathic Arthritis Treated With Canakinumab." 12/01/2013 - "Reactive macrophage activation syndrome possibly triggered by canakinumab in a patient with adult-onset Still's disease." 12/20/2012 - "The macrophage activation syndrome occurred in 7 patients; infections were more frequent with canakinumab than with placebo. "
5.	TNFR-Fc fusion protein (etanercept)FDA Link 10/01/2010 - "The clinical course of this case suggests that the increased TNF-α level by etanercept administration might cause macrophage activation syndrome in this case." 10/01/2010 - "A 44-year-old male patient presented with AOSD complicated by macrophage activation syndrome after etanercept therapy. " 02/01/2008 - "Etanercept for therapy-resistant macrophage activation syndrome." 01/01/2007 - "Macrophage activation syndrome after etanercept treatment." 09/01/2001 - "Etanercept in the treatment of macrophage activation syndrome."
6.	tocilizumabFDA Link 01/01/2013 - "Regarding safety, tocilizumab is a well-tolerated treatment, but severe side effects such as macrophage activation syndrome or cytomegalovirus reactivation are possible and require ongoing vigilance. " 07/01/2015 - "We report a case of macrophage activation syndrome that complicated acute hepatitis E and started within 24hours after the fourth tocilizumab infusion in a patient with RA. " 04/01/2015 - "Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis under Treatment with Tocilizumab." 02/01/2011 - "Benefit and a possible risk of tocilizumab therapy for adult-onset Still's disease accompanied by macrophage-activation syndrome." 01/01/2012 - "Risk of significant cytopenias after treatment with tocilizumab in systemic juvenile arthritis patients with a history of macrophage activation syndrome."
7.	CytokinesIBA 03/01/2012 - "The association with macrophage activation syndrome, and the therapeutic efficacy of inhibiting monocyte-derived cytokines, has implicated these cells in SJIA pathogenesis. " 12/01/2006 - "Macrophage activation syndrome, a life-threatening complication of rheumatic disorders, is accompanied by the overproduction of cytokines. " 04/01/2011 - "Systemic-onset juvenile idiopathic arthritis (systemic JIA) and macrophage activation syndrome (MAS), the most devastating complication of systemic JIA, are characterized by abnormal levels of proinflammatory cytokines. " 10/01/2012 - "To investigate the role of alternative activation of macrophages, in particular, the role of the CD163/HO-1 axis in systemic juvenile idiopathic arthritis (s-JIA), we serially examined the concentrations of HO-1, sCD163 and pro-inflammatory cytokines (IL-10, IL-18, IL-6, neopterin, soluble TNF-α receptor types I and II) in patients with s-JIA complicated by macrophage activation syndrome (MAS/s-JIA). " 09/01/2013 - "Very low density lipoprotein, chylomicron, and lipoprotein (a) are more useful to detect the development of macrophage activation syndrome in adult-onset still's disease as compared with cytokines and triglyceride."
8.	Interleukin-1 (Interleukin 1)IBA 11/01/2014 - "Current guidelines support the early use of a short-acting IL-1 antagonist for macrophage activation syndrome, a life-threatening complication. " 02/01/2011 - "Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients." 01/01/2011 - "These results suggest that early institution of interleukin 1 blockade merits further investigation for the treatment of macrophage activation syndrome and, perhaps, related conditions such as hemophagocytic lymphohistiocytosis." 12/01/2012 - "He developed life-threatening macrophage activation syndrome (MAS), which was refractory to standard immunosuppression but responded dramatically to the IL-1 receptor antagonist anakinra. " 01/01/2014 - "Additionally, interleukin-1 blockade appears to be effective treatment of macrophage activation syndrome, one of the most dangerous complications of JIA; specifically, anakinra in combination with cyclosporine and corticosteroids may obviate the need for cytotoxic approaches. "
9.	infliximab (Remicade)FDA Link 03/01/2011 - "Successful use of infliximab in macrophage activation syndrome with severe CNS involvement." 04/23/2005 - "Elimination of the known causes of macrophage activation syndrome suggests it is related to the infliximab treatment, but its pathogenesis remains a mystery." 04/23/2005 - "Macrophage activation syndrome has never been reported as an adverse effect of infliximab. " 01/01/2013 - "Macrophage activation syndrome revealing disseminated tuberculosis in a patient on infliximab." 04/23/2005 - "[Macrophage activation syndrome after treatment with infliximab for fistulated Crohn's disease]."
10.	Biological Markers (Surrogate Marker)IBA 05/01/2015 - "In systemic JIA, measurement of biomarkers that reflect the degree of activation and expansion of T cells and macrophages might be helpful for detecting subclinical macrophage activation syndrome. " 07/01/2013 - "Our objective was to correlate FSTL-1 levels with gene expression, known biomarkers, and measures of disease activity in systemic juvenile idiopathic arthritis (sJIA), including macrophage activation syndrome (MAS). " 04/01/2015 - "To identify macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA) undergoing tocilizumab (TCZ) treatment, and to confirm laboratory marker changes and responses to treatment in patients with MAS receiving TCZ. " 07/01/2013 - "Follistatin-like protein 1 and the ferritin/erythrocyte sedimentation rate ratio are potential biomarkers for dysregulated gene expression and macrophage activation syndrome in systemic juvenile idiopathic arthritis."

Therapies and Procedures

1.	Splenectomy 03/01/2013 - "Macrophage activation syndrome resistant to medical therapy in a patient with systemic lupus erythematosus and its remission with splenectomy."
2.	Stem Cell Transplantation 10/01/2014 - "Successful stem cell transplantation in a child with chronic granulomatous disease associated with contiguous gene deletion syndrome and complicated by macrophage activation syndrome." 12/01/2002 - "Macrophage activation syndrome after autologous stem cell transplantation for systemic juvenile idiopathic arthritis."
3.	Intensive Care (Surgical Intensive Care) 02/01/2007 - "We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. " 09/01/2012 - "Intensive care requirement, rather than degree of serum ferritin elevation, predicts mortality in macrophage activation syndrome."
4.	Liver Transplantation 11/01/2013 - "Liver transplantation for acute liver failure caused by macrophage activation syndrome." 12/01/2000 - "[Macrophage activation syndrome in primary human herpes virus-6 infection: a rare condition after liver transplantation in infants]."
5.	Plasmapheresis 11/01/2015 - "Successful treatment of macrophage activation syndrome in a patient with dermatomyositis by combination with immunosuppressive therapy and plasmapheresis." 07/01/2012 - "Plasmapheresis should be considered as rescue treatment in patients with life threatening macrophage activation syndrome and systemic onset of juvenile dermatomyositis." 07/01/2012 - "[Plasmapheresis for macrophage activation syndrome and multiorgan failure as first presentation of juvenile dermatomyositis]."