Macrophage activation syndrome: Serological markers and treatment with anti-thymocyte globulin

Coca, Andreea; Bundy, Kemp W.; Marston, Bethany; Huggins, Jennifer; Looney, R. John

doi:10.1016/j.clim.2009.02.005

Cited by 61 publications

(38 citation statements)

References 72 publications

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“…86 A more aggressive approach using anti-thymocyte globulin has been proposed for FHLH 87 and has been used successfully in two patients with probable MAS. 88 Nevertheless, there is a significant risk of serious infection and mortality with anti-thymocyte globulin use, 87 and less aggressive but effective biologic therapies have found recent success in treating MAS as part of sJIA.…”

Section: Traditional Therapy For Mas In the Setting Of Sjiamentioning

confidence: 99%

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

et al. 2012

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Macrophage activation syndrome (MAS) is a severe, frequently fatal complication of systemic juvenile idiopathic arthritis (sJIA) with features of hemophagocytosis leading to coagulopathy, pancytopenia, and liver and central nervous system dysfunction. MAS is overt in 10% of children with sJIA but occurs subclinically in another 30 --40%. It is difficult to distinguish sJIA disease flare from MAS. Development of criteria for establishing MAS as part of sJIA are under way and will hopefully prove sensitive and specific. Mutations in cytolytic pathway genes are increasingly being recognized in children who develop MAS as part of sJIA. Identification of these mutations may someday assist in MAS diagnosis. Defects in cytolytic genes have provided murine models of MAS to study pathophysiology and treatment. Recently, the first mouse model of MAS not requiring infection but rather dependent on repeated stimulation through Toll-like receptors was reported. This provides a model of MAS that may more accurately reflect MAS pathology in the setting of autoinflammation or autoimmunity. This model confirms the importance of a balance between pro-and anti-inflammatory cytokines. There has been remarkable progress in the use of anti-pro-inflammatory cytokine therapy, particularly against interleukin-1, in the treatment of secondary forms of MAS, such as in sJIA.

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Section: Traditional Therapy For Mas In the Setting Of Sjiamentioning

confidence: 99%

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

et al. 2012

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“…Etoposide may also be used to treat MAS but is generally reserved for those who fail corticosteroid and cyclosporine A therapy as this treatment is associated with serious toxicity, including bone marrow suppression and serious infections (33). An alternative therapy to etoposide is antithymocyte globulin; however, this treatment is also less commonly used in those with MAS (34). More recently, the IL-1 blocking agent, anakinra, has been successfully used to treat MAS (35); however, it should be noted that some patients with s-JIA have developed MAS while being managed with anakinra (36).…”

Section: Cytokinesmentioning

confidence: 99%

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Correll

Binstadt

2013

Pediatr Res

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“…Альтернативной и более безопасной цитостатической терапий, по мне-нию A. Coca и соавт. [46], является использование анти-тимоцитарного глобулина, эффективность которого от-мечена у двух пациентов с полиорганной недостаточно-стью (дыхательная недостаточность, печеночная недос-таточность).…”

unclassified

Use of Rituximab in Conjunction With Immunosuppressive Chemotherapy as a Novel Therapy for Epstein Barr Virus-associated Hemophagocytic Lymphohistiocytosis

Balamuth¹,

Nichols²,

Paessler

et al. 2007

Journal of Pediatric Hematology/Oncology

114

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Macrophage activation syndrome: Serological markers and treatment with anti-thymocyte globulin

Cited by 61 publications

References 72 publications

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Use of Rituximab in Conjunction With Immunosuppressive Chemotherapy as a Novel Therapy for Epstein Barr Virus-associated Hemophagocytic Lymphohistiocytosis

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