Treatment of Systemic-onset JIA with Macrophage Activation Syndrome
Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (systemic JIA) in patients under 18 years of age. Up to 40% of systemic JIA cases are associated with MAS — a secondary hemophagocytic syndrome that requires urgent recognition and treatment.
Clinical Scenario
Systemic JIA in a patient under 18 years presenting with active macrophage activation syndrome, identified by the following features:
Fevers
High ferritin
Cytopenias
Elevated liver enzymes
Low fibrinogen
High triglycerides
Treatment Goals
- Inactive disease
- Resolution of fevers
- Normalization of ferritin levels
- Resolution of cytopenias
- Normalization of liver enzyme levels
- Normalization of fibrinogen levels
- Normalization of triglyceride levels
Treatment Approach (Partial)
The protocol involves interleukin-targeted biologic therapy combined with systemic glucocorticoids at the lowest effective dose; further immunosuppressive measures may be added when the initial combination is insufficient to control MAS.
Full regimen, agent selection, and sequencing available in the complete protocol below.
References
DOI: 10.1002/art.42037
- Up to 40% of cases of systemic JIA are associated with MAS, a secondary hemophagocytic syndrome that is a life-threatening complication requiring urgent recognition and treatment.
- MAS presents with fevers, high ferritin levels, cytopenias, elevated liver enzyme levels, low fibrinogen levels, and high triglyceride levels.
- IL-1 or IL-6 inhibitors are conditionally recommended over calcineurin inhibitors alone to achieve inactive disease and resolution of MAS for systemic JIA with MAS.
- There is no preferred agent.
- Glucocorticoids are conditionally recommended as part of initial treatment of systemic JIA with MAS.
- Systemic glucocorticoids may be necessary for severely ill patients because they can have a rapid onset of action.
- Biologic DMARDs combined with glucocorticoids and calcineurin inhibitors may be necessary to control MAS in some patients.
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