Autoinflammatory & rare systemic diseases
Treatment outcomes and predictors of macrophage activation syndrome in adult-onset Still’s disease: a 24 year single-centre experience
Authors' conclusion
In this cohort, IL-1 and IL-6 blockade demonstrated similar effectiveness in AOSD, and biologic switching was followed by remission in several patients. Longer diagnostic delay was associated with less favourable treatment outcomes. Elevated baseline ferritin and systemic scores were associated with MAS.
Study context
- Population
- Adults diagnosed with Adult-onset Still's disease (AOSD) (Yamaguchi criteria) between 2000 and 2024
- Participants
- 93 adults
- Exposure
- Anakinra vs Tocilizumab
- Outcome
- Treatment response and factors associated with MAS
Methodological detailssingle-centre, multivariable logistic regression, ROC curve analysis
- Single-centre
- Multivariable logistic regression
- ROC curve analysis
Main finding
As reported in the abstract
- Higher baseline ferritin independently associated with macrophage activation syndrome (MAS), OR
- 2.18 (95% CI 1.12-4.24)
- Higher systemic scores independently associated with MAS, OR
- 2.52 (95% CI 1.28-4.96)
- Baseline ferritin discrimination for MAS, AUC
- 0.82 (95% CI 0.68-0.95; p < 0.001)
- Ferritin cut-off for MAS
- 2000 ng/mL
- Systemic score ≥ 7 associated with MAS, OR
- 18.27 (95% CI 4.25-78.47)
Observational data: associations, not effects.
Further findings
Tocilizumab after anakinra non-response
- Anakinra-unresponsive patients
- 17
- Received tocilizumab
- 16
- Achieved complete remission
- 11
Diagnostic delay and treatment outcomes
- Diagnostic delay linked to poorer MTX response
- P = 0.0457
- Diagnostic delay linked to lower rates of GC discontinuation
- P < 0.01
Original publication
Treatment outcomes and predictors of macrophage activation syndrome in adult-onset Still’s disease: a 24 year single-centre experience. Scand J Rheumatol. Published 28 April 2026. doi:10.
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