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Macrophage Activation Syndrome in Rheumatic Diseases

  • Alexei A. GromEmail author
  • Edward M. Behrens
Chapter

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.

Keywords

Macrophage activation syndrome Systemic juvenile idiopathic arthritis Still disease Hemophagocytic macrophages Hemophagocytic lymphohistiocytosis 

Abbreviations

AOSD

Adult onset Still disease

ATG

Anti-thymocyte globulin

BM

Bone marrow

CMV

Cytomegalovirus

CRP

C-reactive protein

DIC

Disseminated intravascular coagulation

EBV

Epstein Barr virus

ESR

Erythrocyte sedimentation rate

HLH

Hemophagocytic lymphohistiocytosis

IRF5

Interferon regulatory factor 5

MAS

Macrophage activation syndrome

NK cells

Natural killer cells

SJIA

Systemic juvenile idiopathic arthritis

TLR

Toll-like receptor

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Cincinnati Children’s Hospital Medical CenterCincinnatiUSA
  2. 2.Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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