Clinical features and correct diagnosis of macrophage activation syndrome

Expert Rev Clin Immunol. 2015;11(9):1043-53. doi: 10.1586/1744666X.2015.1058159. Epub 2015 Jun 16.


Macrophage activation syndrome (MAS) is increasingly recognized among febrile hospitalized patients. Clinically, MAS resembles multiorgan dysfunction and shock. Laboratory features include hepatobiliary dysfunction, coagulopathy, pancytopenia, hyperferritinemia and markers of immune activation. Pathologically, hemophagocytosis is commonly seen but is only present in 60% of MAS patients. MAS, or secondary hemophagocytic lymphohistiocytosis (HLH), is triggered by infectious (e.g., herpes family viruses), rheumatologic (e.g., systemic lupus erythematosus [SLE]) and oncologic (e.g., T-cell leukemia) conditions. Formal HLH criteria, while specific, are frequently insensitive for MAS diagnosis. Thus, disease-specific (e.g., SLE) and generic MAS criteria have been published. Recently, novel criteria for MAS in children with systemic juvenile idiopathic arthritis (sJIA) were developed and are a key focus of this review.

Keywords: coagulopathy; fever; hemophagocytic lymphohistiocytois; hemophagocytosis; hyperferritinemia; macrophage activation syndrome; multi-organ dysfunction; systemic juvenile idiopathic arthritis.

Publication types

  • Review

MeSH terms

  • Adult
  • Arthritis, Juvenile / complications
  • Arthritis, Juvenile / immunology*
  • Bacterial Infections / complications
  • Bacterial Infections / immunology*
  • Child
  • Early Diagnosis
  • Humans
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / immunology*
  • Macrophage Activation Syndrome / complications
  • Macrophage Activation Syndrome / diagnosis
  • Macrophage Activation Syndrome / immunology*
  • Neoplasms / complications
  • Neoplasms / immunology*
  • Virus Diseases / complications
  • Virus Diseases / immunology*