Lupus & antiphospholipid syndrome
Macrophage activation syndrome is associated with high in-hospital mortality in patients with lupus
Authors' conclusion
MAS is a rare complication of active lupus identified by fever, elevated ferritin, liver transaminases and cytopenia and is associated with high in hospital mortality.
Study context
- Population
- Hospitalised adult-onset SLE patients diagnosed with MAS fulfilling the Parodi criteria with ferritin >500 ng/ml, with all other hospitalised SLE patients without MAS as controlsSLE patients with 623 hospitalisations
- Participants
- 451 patients
- Exposure
- SLE patients with MAS vs hospitalised SLE patients without MAS
- MASn = 86
- CONTROLSn = 526
- Outcome
- MAS at the time of SLE diagnosis
Methodological detailstertiary care referral centre, 2017 to 2022, consecutive, univariate and multivariate analyses with a stepwise logistic regression
- Tertiary care referral centre
- 2017 to 2022
- Consecutive
- Univariate and multivariate analyses with a stepwise logistic regression
Main finding
As reported in the abstract
MAS
62.9%
54
Observational data: associations, not effects.
Further findings
In-hospital mortality · higher in MAS
P < 0.001
| Outcome | MAS | CONTROLS | P value |
|---|---|---|---|
| Disease activity (clinical SLEDAI 2K)Significantly higher in MAS | mean 13.7 ± 6.5 | mean 8 ± 5.9 | P < 0.001 |
Clinical features associated with MAS
- MAS was associated with fever, hepatosplenomegaly, lymphadenopathy, cytopenia, and elevated transaminases
Factors associated with mortality in MAS (univariate)
- Ferritin, died vs survived
- 12092.9 ± 2826.5 vs 3993.6 ± 735, <0.001
- Aspartate aminotransferase, died vs survived
- 544 ± 239 vs 167.66 ± 27.28U/L, p < 0.001
- Seizures
- P = 0.035
- Altered mental status
- P = 0.004
- Shock
- P < 0.001
- Intensive care unit admission
- P < 0.001
- Mechanical ventilation
- P < 0.001
Mortality in MAS, multivariate regression
- Ferritin >2400, OR
- 8.1 95% CI: (1.46-44.85) p - 0.017
- Seizures, OR
- 7.3 95% CI: (1.2-44.29) p - 0.030
Original publication
Macrophage activation syndrome is associated with high in-hospital mortality in patients with lupus. Lupus. Published 23 October 2026. doi:10. 1177/ 09612033261500970
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