Lupus & antiphospholipid syndrome

Macrophage activation syndrome is associated with high in-hospital mortality in patients with lupus

LupusPublished retrospective study

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

OutcomeMASCONTROLSP value
Disease activity (clinical SLEDAI 2K)Significantly higher in MASmean 13.7 ± 6.5mean 8 ± 5.9P < 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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