title: Therapeutic plasma exchange in chronic dysimmune peripheral neuropathies: A 10-year retrospective study.
abstract: BACKGROUND
  Therapeutic plasma exchange (TPE) can be proposed in the treatment of chronic dysimmune peripheral neuropathies (CDPN). Actual guidelines are however based on few studies, and indications and protocols still remain to be clarified. We conducted a 10-year retrospective study in order to assess the effectiveness and tolerance of TPE in CDPN.
  
  
  METHODS
  All patients treated for CDPN with TPE from October 2006 to March 2016 in the university hospital of Angers were included. Patients were considered responders when they presented a clinical improvement substantial enough to continue the treatment. The Hughes functional grading score was also determined for each patient before and after TPE initiation.
  
  
  RESULTS
  Among the 206 patients who received TPE during the study period, 30 (14.6%) met the diagnostic criteria of CDPN. Four of the five paraprotein neuropathies (PPN) patients (80%) and 8 of the 11 chronic inflammatory demyelinating polyneuropathies (CIDP) patients (72.7%) were responders, with a significant improvement of the Hughes score for the latter (P = 0.013). None of the three Lewis-Sumner and the two POEMS patients showed substantial improvement. Six of the nine anti-MAG neuropathy patients (66.7%) responded to treatment, with a trend towards improvement of the Hughes score (P = 0.072).
  
  
  CONCLUSIONS
  TPE appears to be effective in CIDP and PPN, and ineffective in Lewis-Sumner and POEMS syndromes. Interestingly, anti-MAG neuropathy patients showed a good rate of response to TPE. Regarding these preliminary results, a randomized trial would be very worthwhile in this disease for which there is no evidence based treatment to date.
authors.affiliation: Department of Neurology, University Hospital of Angers, Angers, France.|Reference Centre for Neuromuscular Diseases, Department of Neurology, University Hospital of Angers, Angers, France.|Department of Neurobiology and Neuropathology, University Hospital of Angers, Angers, France.|Department of Nephrology, University Hospital of Angers, Angers, France.
authors.forename: Philippe
authors.identifier: http://orcid.org/0000-0003-1745-8299
authors.initials: P
authors.lastname: Codron
authors.affiliation: School of Medicine, University of Angers, Angers, France.
authors.forename: Maud
authors.initials: M
authors.lastname: Cousin
authors.affiliation: School of Medicine, University of Angers, Angers, France.|Laboratory of Integrated Neurovascular and Mitochondrial Biology (CNRS 6214/INSERM 1083), University Hospital of Angers, Angers, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Subra
authors.affiliation: Department of Neurology, University Hospital of Angers, Angers, France.|Reference Centre for Neuromuscular Diseases, Department of Neurology, University Hospital of Angers, Angers, France.
authors.forename: Vivien
authors.initials: V
authors.lastname: Pautot
authors.affiliation: Reference Centre for Neuromuscular Diseases, Department of Neurology, University Hospital of Angers, Angers, France.|Department of Neurobiology and Neuropathology, University Hospital of Angers, Angers, France.|Laboratory of Integrated Neurovascular and Mitochondrial Biology (CNRS 6214/INSERM 1083), University Hospital of Angers, Angers, France.
authors.forename: Franck
authors.initials: F
authors.lastname: Letournel
authors.affiliation: Department of Neurology, University Hospital of Angers, Angers, France.|Department of Nephrology, University Hospital of Angers, Angers, France.|Laboratory of Integrated Neurovascular and Mitochondrial Biology (CNRS 6214/INSERM 1083), University Hospital of Angers, Angers, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Verny
authors.affiliation: Department of Neurology, University Hospital of Angers, Angers, France.|Reference Centre for Neuromuscular Diseases, Department of Neurology, University Hospital of Angers, Angers, France.|Department of Nephrology, University Hospital of Angers, Angers, France.|Laboratory of Integrated Neurovascular and Mitochondrial Biology (CNRS 6214/INSERM 1083), University Hospital of Angers, Angers, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Cassereau
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000628:therapy; D010951:Plasma Exchange / Q000379:methods*; D011115:Polyneuropathies / Q000628:therapy*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000628:therapy; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: anti-MAG neuropathy; chronic dysimmune neuropathy; chronic inflammatory demyelinating polyneuropathies; paraproteinemic polyneuropathy; therapeutic plasma exchange