Autoinflammatory & rare systemic diseases
Diagnostic performance of HScore for macrophage activation syndrome complicating Still’s disease in adults: a multicentre case–control study
Authors' conclusion
The HScore and modified HScore could be useful tools for diagnosing SD-MAS in adults with and without bone marrow aspiration, respectively.
Study context
- Population
- Patients ≥16 years with active Still's disease, three medical centres in Japan, 2004–2023; allocated to SD without macrophage activation syndrome (MAS) and SD-MAS by an expert panel25 with SD-MAS
- Participants
- 86 patients
- Comparison
- HScore vs HLH-04, MAS (MAS-2016) and modified HScore for diagnosing SD-MAS in adults
- Outcome
- Diagnostic performance (ROC discriminative ability) for SD-MAS
Methodological detailscase-control, multicentre, ROC curve analysis, logistic regression
- Case-control
- Multicentre
- ROC curve analysis
- Logistic regression
Performance
As reported in the abstract
Discrimination of SD-MAS
| Test or model | AUC | Cut-off | Sensitivity | Specificity |
|---|---|---|---|---|
| HScore | 0.991 (95% CI 0.977-1.000) | 192 | 96.0% | 96.7% |
| Modified HScore | 171 | 92.0% | 83.6% |
AUC
HScore0.991 (95% CI 0.977-1.000)
Modified HScore— not stated in the abstract
Cut-off
HScore192
Modified HScore171
Sensitivity
HScore96.0%
Modified HScore92.0%
Specificity
HScore96.7%
Modified HScore83.6%
Further findings
Multivariate logistic regression, model-1 (HScore and HLH-04)
| OR (95% CI) | ||
|---|---|---|
| HScore | 1.271 (1.006–1.606) | |
| 0.512Odds ratio (log scale) |
Multivariate logistic regression, model-2 (MAS-2016 and modified HScore)
| OR (95% CI) | ||
|---|---|---|
| MAS-2016 | 4.249 (1.636–11.041) | |
| Modified HScore | 1.066 (1.017–1.118) | |
| 0.050.21520Odds ratio (log scale) |
Multivariate logistic regression, model-1 (HScore and HLH-04)
- Only HScore identified in model-1
Original publication
Diagnostic performance of HScore for macrophage activation syndrome complicating Still’s disease in adults: a multicentre case–control study. Rheumatology (Oxford). Published 31 August 2026. doi:10.
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