Macrophage activation syndrome (MAS) is a recognised complication of Adult-onset Still disease. When it occurs, it is identified by a characteristic combination of clinical and laboratory findings that indicate a specific and intensive treatment approach.
MAS should be considered in patients with Adult-onset Still disease who present with persistent fever alongside splenomegaly, elevated or rising serum ferritin, inappropriately low cell counts, abnormal liver function tests, intravascular activation of coagulation, and elevated or rising serum triglycerides.
High-dose glucocorticoids are the mainstay of MAS treatment, and combination immunosuppressive agents are considered as part of initial therapy alongside targeted management of any identified triggering infection.
Full regimen, agent selection, sequencing, and dosing are available in the complete structured protocol →MAS should be considered in patients with persistent fever, splenomegaly, elevated or rising serum ferritin, inappropriately low cell counts, abnormal LFTs, intravascular activation of coagulation, elevated or rising serum triglycerides.
MAS treatment must include high dose GCs. In addition, treatments including anakinra, ciclosporin and/or IFNgamma inhibitors should be considered as part of initial therapy.
DOI: 10.1136/ard-2024-225851
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