Initial diagnosis of Macrophage Activation Syndrome
GGC Paediatric Guidelines
Warning
Macrophage activation syndrome (MAS) is a rare hyperinflammatory condition, one of the increasingly-recognised cytokine storms, seen in children and adults with underlying rheumatological disorders. MAS requires prompt recognition and aggressive treatment as untreated the mortality rate is 8-22%.
This guideline focusses on MAS in the context of rheumatological disorders. If the child does not have a known underlying diagnosis, the differential diagnoses of primary or secondary HLH and MAS as part of the initial presentation of a rheumatological disorder should be considered.
Contents:
- Definition
- Urgent Investigations
- Published Diagnostic & Classification Criteria
- Initial Treatment
- Additional Considerations