Lupus & antiphospholipid syndrome
Macrophage activation syndrome in systemic lupus erythematous and Still’s disease: distinct phenotypes shaped by the underlying diseases
Authors' conclusion
SLE-MAS diverged from SD-MAS with less organomegaly and liver injury but more pronounced CNS involvement and hematological involvement, the latter strongly linked to red blood cell directed autoantibodies.
Study context
- Population
- Adults with a history of SLE-MAS or SD-MAS, eight tertiary referral centres
- Exposure
- SLE-MAS vs SD-MAS
- Outcome
- MAS clinical/laboratory features, treatments and outcomes compared between SLE-MAS and SD-MAS
Methodological detailsmulticentre, eight tertiary referral centres, exploratory multivariable profiling, comparison with historical SLE cohorts
- Multicentre
- Eight tertiary referral centres
- Exploratory multivariable profiling
- Comparison with historical SLE cohorts
Main finding
As reported in the abstract
MAS clinical/laboratory features
Meeting 2016 ACR/EULAR/PRINTO criteria
| Outcome | SLE-MAS | SD-MAS |
|---|---|---|
| Direct Coombs (DCT) positivity | 73% | — not stated in the abstract |
— not stated in the abstract
Observational data: associations, not effects.
Further findings
| Outcome | Values |
|---|---|
| IL-1 antagonism in SLE-MAS | SLE-MAS 7 events |
IL-1 antagonism in SLE-MAS
- Mixed results without any rapid macrophage activation syndrome (MAS) resolution
SLE-MAS vs historical SLE cohorts (1000, 2228 and 2055 cases)
- Mucocutaneous disease, autoimmune haemolysis, CNS involvement, less pronounced female preponderance and much higher DCT positivity dominated the SLE-MAS phenotype
Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.
Original publication
Macrophage activation syndrome in systemic lupus erythematous and Still’s disease: distinct phenotypes shaped by the underlying diseases. Rheumatology (Oxford). Published 11 September 2026. doi:10.
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