title: Plasma exchange in pediatric anti-NMDAR encephalitis: A systematic review.
abstract: OBJECTIVE
  To clarify the most frequent modalities of use of plasma exchange (PE) in pediatric anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis and to establish the most effective association with other immunotherapies.
  
  
  METHODS
  Systematic literature review on PE in pediatric anti-NMDAR encephalitis (2007-2015).
  
  
  RESULTS
  Seventy-one articles were included (mostly retrospective), reporting a total of 242 subjects (73.2%, 93/127 females; median age at onset 12years, range 1-18). Median time to immunotherapy was 21days (range 0-190). In most cases, PE was given with steroids and IVIG (69.5%, 89/128), or steroids only (18%, 23/128); in a minority, it was associated with IVIG only (7%, 9/128), or was the only first-line treatment (5.5%, 7/128). In 54.5% (65/119), PE was the third treatment after steroids and IVIG, in 31.1% (37/119) the second after steroids or IVIG; only in 14.3% (17/119) was it the first treatment. Second-line immunotherapies were administered in 71.9% (100/139). Higher rates of full/substantial recovery at follow-up were observed with immunotherapy given ⩽30days from onset (69.4%, 25/36) compared to later (59.2%, 16/27), and when PE was associated with steroids (66.7%, 70/105) rather than not (46.7%, 7/15). Significant adverse reactions to PE were reported in 6 patients.
  
  
  CONCLUSIONS
  Our review disclosed a paucity of quality data on PE in pediatric anti-NMDAR encephalitis. PE use in this condition has been increasingly reported, most often with steroids and IVIG. Despite the limited number of patients, our data seem to confirm the trend towards a better outcome when PE was administered early, and when given with steroids.
authors.affiliation: Pediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Italy. Electronic address: suppiej@pediatria.unipd.it.
authors.forename: Agnese
authors.initials: A
authors.lastname: Suppiej
authors.affiliation: Pediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Nosadini
authors.affiliation: Department of Neurology, Ospedale Ca' Foncello, Treviso, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Zuliani
authors.affiliation: Pediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Italy.
authors.forename: Maria Federica
authors.initials: MF
authors.lastname: Pelizza
authors.affiliation: Pediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Italy.
authors.forename: Irene
authors.initials: I
authors.lastname: Toldo
authors.affiliation: Pediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Bertossi
authors.affiliation: Immunotransfusion Section, University Hospital of Padua, Italy.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Tison
authors.affiliation: Department of Neurology, Ospedale Ca' Foncello, Treviso, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Zoccarato
authors.affiliation: Immunotransfusion Section, University Hospital of Padua, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Marson
authors.affiliation: Department of Neurology, Ospedale Ca' Foncello, Treviso, Italy.
authors.forename: Bruno
authors.initials: B
authors.lastname: Giometto
authors.affiliation: Neuroimmunology group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Australia.
authors.forename: Russell C
authors.initials: RC
authors.lastname: Dale
authors.affiliation: Pediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Sartori
mesh_terms: D000293:Adolescent; D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000453:epidemiology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D010951:Plasma Exchange / Q000379:methods* / Q000706:statistics & numerical data
keywords: Anti-NMDAR; Apheresis; Children; Encephalitis; Immune therapy; Plasma exchange; Plasmapheresis