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RESEARCH PRODUCT
Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis
Nicolino RupertoAntonella BuoncompagniBianca LattanziZane DavidsoneMaka IoselianiJaime De InocencioCarmen De CuntoJeffrey M. LiptonAngelo RavelliClaudia Saad MagalhãesSandra EncisoThomas A. GriffinPatrizia BaroneHasan TezerMichael JengFrancesca BovisPaolo PiccoSulaiman M. Al-mayoufAgeza M KapovicNuray Aktay AyazJonathan D AkikusaErkan DemirkayaIngrida Rumba-rozenfeldeSilvia Magni ManzoniOlga VougioukaB. BicaAlberto MartiniRandy Q. CronRomina GallizziFrancesca MinoiaIsabel BoltWafaa SewairiLuciana BredaAnnacarin HorneSergio DavìTeresa HennonG. HorneffLehn K. WeaverKimo C. Stinesubject
MaleHEMOPHAGOCYTIC SYNDROMESInternationalityDatabases FactualHepatosplenomegalyJuvenileComorbidityHEMOPHAGOCYTIC LYMPHOHISTIOCYTOSISSeverity of Illness IndexHEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HEMOPHAGOCYTIC SYNDROMES; MACROPHAGE ACTIVATION SYNDROME; SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS; Adolescent; Age Distribution; Arthritis Juvenile; Child; Child Preschool; Cohort Studies; Comorbidity; Databases Factual; Female; Humans; Internationality; Macrophage Activation Syndrome; Male; Multivariate Analysis; Prevalence; Prognosis; Retrospective Studies; Severity of Illness Index; Sex Distribution; Survival Analysis; Immunology and Allergy; Rheumatology; ImmunologyCohort StudiesPrevalenceImmunology and AllergyChildMacrophage Activation SyndromePrognosisChild PreschoolFemaleHemophagocytosismedicine.symptommedicine.medical_specialtyAdolescentSYSTEMIC JUVENILE IDIOPATHIC ARTHRITISImmunologyHemophagocytic Lymphohistiocytosis Hemophagocytic Syndromes Macrophage activation syndromes Systemic Juvenile Idiopathic Arthritis Adolescent Age Distribution Arthritis Juvenile Child Child Preschool Cohort Studies Comorbidity Databases Factual Female Humans Internationality Macrophage Activation Syndrome Male Multivariate Analysis Prevalence Prognosis Retrospective Studies Severity of Illness Index Sex Distribution Survival Analysis Immunology and Allergy Rheumatology ImmunologyDatabasesAge DistributionRheumatologyInternal medicineSeverity of illnessmedicineHumansSex DistributionPreschoolFactualRetrospective StudiesHemophagocytic lymphohistiocytosisbusiness.industryArthritisRetrospective cohort studymedicine.diseaseSurvival AnalysisComorbidityArthritis JuvenileRheumatologyMacrophage activation syndromeMultivariate AnalysisImmunologyMacrophage activation syndromesbusinessdescription
Objective.To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey.Methods.International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course.Results.A total of 362 patients were included by 95 investigators from 33 countries. Demographic, clinical, laboratory, and histopathologic features were comparable among patients seen in diverse geographic areas or by different pediatric specialists. Patients seen in North America were given biologics more frequently. Patients entered by pediatric hemato-oncologists were treated more commonly with biologics and etoposide, whereas patients seen by pediatric rheumatologists more frequently received cyclosporine. Patients with demonstration of hemophagocytosis had shorter duration of sJIA at MAS onset, higher prevalence of hepatosplenomegaly, lower levels of platelets and fibrinogen, and were more frequently administered cyclosporine, intravenous immunoglobulin (IVIG), and etoposide. Patients with severe course were older, had longer duration of sJIA at MAS onset, had more full-blown clinical picture, and were more commonly given cyclosporine, IVIG, and etoposide.Conclusion.The clinical spectrum of MAS is comparable across patients seen in different geographic settings or by diverse pediatric subspecialists. There was a disparity in the therapeutic choices among physicians that underscores the need to establish uniform therapeutic protocols.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2015-01-01 | The Journal of Rheumatology |