Macrophage Activation Syndrome in MIS-C

Gámez-González L.B.; Murata C.; García-Silva J.; Ulloa-Gutierrez R.; Márquez-Aguirre M.; Ríos-Olivares I.; Faugier-Fuentes E.; Domínguez-Rojas J.A.; Yock-Corrales A.; Álvarez-Olmos M.I.; Fernández-Sarmiento J.; Velasquez-Méndez M.; Ivankovich-Escoto G.; Tremoulet A.H.; Yamazaki-Nakashimada M.A.; Fabi M.; Galvez-Rafael N.; Padilla T.; Villarreal-Treviño A.V.; del Águila O.; Llamas-Guillén B.A.; Criales J.; Chacon-Cruz E.; García-Domínguez M.; Gatica A.; Cofré F.; Rubio-Pérez N.E.; Santana-Chalas G.M.; García-Aguilar H.; Zapata Yarlequé E.H.; Arbo A.; Lovera D.; Cuan S.; Díaz A.; Franco L.; Guarnizo P.; Boza R.; Huertas-Quiñones M.; Medina-Ramos D.C.; Lozano-Jaramillo M.I.; Aguilera-Martínez S.I.; Fajardo-Latorre V.A.; Morales-Burton V.; Paternina-De la Ossa R.A.; Rojas J.P.; Moreno E.; García-Rodríguez F.; Bravo-López M.G.; González-Mata A.; Buonsenso D. · Pediatrics (2024) · DOI: 10.1542/peds.2024-066780

Resumen

BACKGROUND: Multisystem inflammatory syndrome (MIS-C) represents a diagnostic challenge because of its overlap with Kawasaki disease, Kawasaki disease shock syndrome, and toxic shock syndrome. Macrophage activation syndrome (MAS) is a frequently fatal complication of various pediatric inflammatory disorders and has been reported in MIS-C. Early diagnosis and prompt initiation by immune modulating therapies are essential for effectively managing MAS. METHODS: We conducted a retrospective study to determine the frequency, natural history, diagnostic metrics, treatment, and outcome of MAS in MIS-C within a large cohort of patients across 84 Latin American centers in 16 countries. We compared the clinical and laboratory characteristics between patients with and without MAS. RESULTS: Among 1238 patients with MIS-C, 212 (17.1%) fulfilled MAS criteria. Gastrointestinal and neurologic manifestations were more frequent in cases where MIS-C was complicated by MAS. Patients presenting with MIS-C complicated by MAS had a mortality rate of 12%, which was higher than those without it. Mortality was associated with MAS, seizures, arthritis, and shock. A ferritin or erythrocyte sedimentation rate ratio of >18.7 exhibited a sensitivity of 88.2% and a specificity of 75% in diagnosing MAS in MIS-C. CONCLUSIONS: MAS in MIS-C patients is associated with increased morbidity and mortality rates in the largest MIS-C Latin American cohort. Early recognition and appropriate management are crucial in improving patient outcomes and reducing mortality rates. Copyright