title: Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients.
abstract: OBJECTIVE
  To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).
  
  
  METHODS
  In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database.
  
  
  RESULTS
  A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and d-dimer levels were the sole laboratory biomarkers showing a percentage change of >50% between the pre-MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids, and 61% received cyclosporine. Biologic medications and etoposide were given to 15% and 12% of the patients, respectively. Approximately one-third of the patients required admission to the intensive care unit (ICU), and the mortality rate was 8%.
  
  
  CONCLUSIONS
  This study provides information on the clinical spectrum and current management of systemic JIA-associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.
authors: Istituto Giannina Gaslini, Genoa, Italy.
authors: Francesca
authors: F
authors: Minoia
authors: Sergio
authors: S
authors: Davì
authors: AnnaCarin
authors: A
authors: Horne
authors: Erkan
authors: E
authors: Demirkaya
authors: Francesca
authors: F
authors: Bovis
authors: Caifeng
authors: C
authors: Li
authors: Kai
authors: K
authors: Lehmberg
authors: Sheila
authors: S
authors: Weitzman
authors: Antonella
authors: A
authors: Insalaco
authors: Carine
authors: C
authors: Wouters
authors: Susan
authors: S
authors: Shenoi
authors: Graciela
authors: G
authors: Espada
authors: Seza
authors: S
authors: Ozen
authors: Jordi
authors: J
authors: Anton
authors: Raju
authors: R
authors: Khubchandani
authors: Ricardo
authors: R
authors: Russo
authors: Priyankar
authors: P
authors: Pal
authors: Ozgur
authors: O
authors: Kasapcopur
authors: Paivi
authors: P
authors: Miettunen
authors: Despoina
authors: D
authors: Maritsi
authors: Rosa
authors: R
authors: Merino
authors: Bita
authors: B
authors: Shakoory
authors: Maria
authors: M
authors: Alessio
authors: Vyacheslav
authors: V
authors: Chasnyk
authors: Helga
authors: H
authors: Sanner
authors: Yi-Jin
authors: YJ
authors: Gao
authors: Zeng
authors: Z
authors: Huasong
authors: Toshiyuki
authors: T
authors: Kitoh
authors: Tadej
authors: T
authors: Avcin
authors: Michel
authors: M
authors: Fischbach
authors: Michael
authors: M
authors: Frosch
authors: Alexei
authors: A
authors: Grom
authors: Adam
authors: A
authors: Huber
authors: Marija
authors: M
authors: Jelusic
authors: Sujata
authors: S
authors: Sawhney
authors: Yosef
authors: Y
authors: Uziel
authors: Nicolino
authors: N
authors: Ruperto
authors: Alberto
authors: A
authors: Martini
authors: Randy Q
authors: RQ
authors: Cron
authors: Angelo
authors: A
authors: Ravelli
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001171:Arthritis, Juvenile / Q000150:complications*; D001688:Biological Products / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016572:Cyclosporine / Q000627:therapeutic use*; D005047:Etoposide / Q000627:therapeutic use*; D005260:Female; D005334:Fever / Q000453:epidemiology; D006529:Hepatomegaly / Q000453:epidemiology; D006801:Humans; D007362:Intensive Care Units / Q000706:statistics & numerical data; D007391:International Cooperation; D055501:Macrophage Activation Syndrome / Q000188:drug therapy* / Q000209:etiology* / Q000401:mortality; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D013163:Splenomegaly / Q000453:epidemiology; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001688:Biological Products; D005047:Etoposide; D016572:Cyclosporine