AMETHYST : A Retrospective Cohort Study of Treatment Patterns and Outcomes in Patients With Glucocorticoid‐Refractory Macrophage Activation Syndrome Complicating Still's Disease
Study context
- Population
- Patients with glucocorticoid (GC)-refractory macrophage activation syndrome (MAS) complicating Still's disease (sJIA and AOSD); index MAS episodes January 1, 2012, to September 30, 202255 sJIA, 9 AOSD
- Participants
- 64 patients
- Outcome
- Real-world treatment patterns and outcomes in GC-refractory MAS complicating Still's disease
Methodological detailsmulticentre (eight sites in Europe, Canada, and the United States), chart abstraction
- Multicentre (eight sites in Europe, Canada, and the United States)
- Chart abstraction
Main finding
As reported in the abstract
Outcomes of index MAS episode
- Complete MAS laboratory remission (normalisation of seven prespecified lab parameters)
- 7 of 64 (10.9%)
- Partial MAS laboratory remission (normalisation of at least three prespecified lab parameters)
- 41 of 64 (64.1%)
- Complete clinical response (investigator assessment)
- 24 of 64 (37.5%)
- Partial clinical response (investigator assessment)
- 26 of 64 (40.6%)
- GCs tapered
- 50 of 64 (78.1%)
- Median time of GC taper
- 39.9 days
- MAS recurrence
- 20 of 64 (31.3%)
Observational data: associations, not effects.
Further findings
Treatment patterns
- Received GCs
- All patients
- Anakinra
- 48/64 [75%]
- Ciclosporin
- 33/64 [51.6%]
MAS features
- Rash
- 60.4%
- Fever
- 52.8%
- Hepatic involvement
- 49.1%
Death
- Died
- 7 of 64 (10.9%)
- Estimated one-year survival probability
- 93.75%
Original publication
AMETHYST : A Retrospective Cohort Study of Treatment Patterns and Outcomes in Patients With Glucocorticoid‐Refractory Macrophage Activation Syndrome Complicating Still's Disease. Arthritis Rheumatol. Published 7 September 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →