PMID- 31355323
OWN - NLM
STAT- MEDLINE
DCOM- 20200924
LR  - 20240229
IS  - 2332-7812 (Electronic)
IS  - 2332-7812 (Linking)
VI  - 6
IP  - 5
DP  - 2019 Sep
TI  - Long-term safety and efficacy of subcutaneous immunoglobulin IgPro20 in CIDP: 
      PATH extension study.
PG  - e590
LID - 10.1212/NXI.0000000000000590 [doi]
LID - e590
AB  - OBJECTIVE: To investigate the long-term safety and efficacy of weekly 
      subcutaneous IgPro20 (Hizentra, CSL Behring) in chronic inflammatory 
      demyelinating polyneuropathy (CIDP). METHODS: In a 48-week open-label prospective 
      extension study to the PATH study, patients were initially started on 0.2 g/kg or 
      on 0.4 g/kg weekly and-if clinically stable-switched to 0.2 g/kg weekly after 24 
      weeks. Upon CIDP relapse on the 0.2 g/kg dose, 0.4 g/kg was (re)initiated. CIDP 
      relapse was defined as a deterioration by at least 1 point in the total adjusted 
      Inflammatory Neuropathy Cause and Treatment score. RESULTS: Eighty-two patients 
      were enrolled. Sixty-two patients initially received 0.4 g/kg, 20 patients 0.2 
      g/kg weekly. Seventy-two received both doses during the study. Sixty-six patients 
      (81%) completed the 48-week study duration. Overall relapse rates were 10% in 0.4 
      g/kg-treated patients and 48% in 0.2 g/kg-treated patients. After dose reduction 
      from 0.4 to 0.2 g/kg, 51% (27/53) of patients relapsed, of whom 92% (24 of 26) 
      improved after reinitiation of the 0.4 g/kg dose. Two-thirds of patients (19/28) 
      who completed the PATH study without relapse remained relapse-free on the 0.2 
      g/kg dose after dose reduction in the extension study. Sixty-two patients had 
      adverse events (AEs) (76%), of which most were mild or moderate with no related 
      serious AEs. CONCLUSIONS: Subcutaneous treatment with IgPro20 provided long-term 
      benefit at both 0.4 and 0.2 g/kg weekly doses with lower relapse rates on the 
      higher dose. Long-term dosing should be individualized to find the most 
      appropriate dose in a given patient. CLASSIFICATION OF EVIDENCE: This study 
      provides Class IV evidence that for patients with CIDP, long-term treatment with 
      SCIG beyond 24 weeks is safe and efficacious.
FAU - van Schaik, Ivo N
AU  - van Schaik IN
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Mielke, Orell
AU  - Mielke O
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Bril, Vera
AU  - Bril V
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - van Geloven, Nan
AU  - van Geloven N
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Hartung, Hans-Peter
AU  - Hartung HP
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Lewis, Richard A
AU  - Lewis RA
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Sobue, Gen
AU  - Sobue G
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Lawo, John-Philip
AU  - Lawo JP
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Praus, Michaela
AU  - Praus M
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Durn, Billie L
AU  - Durn BL
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Cornblath, David R
AU  - Cornblath DR
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
FAU - Merkies, Ingemar S J
AU  - Merkies ISJ
AD  - Department of Neurology (I.N.S.), Amsterdam University Medical Centers, 
      University of Amsterdam, The Netherlands; Spaarne Gasthuis (I.N.S.), Haarlem, The 
      Netherlands; CSL Behring (O.M., M.P., B.L.D.), Marburg, Germany and King of 
      Prussia, PA; Department of Medicine (Neurology) (V.B.), University Health 
      Network, University of Toronto, Canada; Medical Statistics (N.G.), Department of 
      Biomedical Data Sciences, Leiden University Medical Center, The Netherlands; 
      Department of Neurology (H.-P.H.), Medical Faculty, Heinrich Heine University, 
      Dusseldorf, Germany; Department of Neurology (R.A.L.), Cedars-Sinai Medical 
      Center, Los Angeles, CA; Department of Neurology (G.S.), Nagoya University 
      Graduate School of Medicine, Japan; Department of Neurology (D.R.C.), Johns 
      Hopkins University School of Medicine, Baltimore, MD; and Department of Neurology 
      (I.S.J.M.), Maastricht University Medical Center, The Netherlands.
CN  - PATH study group
LA  - eng
PT  - Journal Article
PT  - Randomized Controlled Trial
PT  - Research Support, Non-U.S. Gov't
DEP - 20190703
PL  - United States
TA  - Neurol Neuroimmunol Neuroinflamm
JT  - Neurology(R) neuroimmunology & neuroinflammation
JID - 101636388
RN  - 0 (Hizentra)
RN  - 0 (Immunoglobulin G)
RN  - 0 (Immunoglobulins, Intravenous)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunoglobulin G/*administration & dosage/adverse effects
MH  - Immunoglobulins, Intravenous/*administration & dosage/adverse effects
MH  - Injections, Subcutaneous
MH  - Male
MH  - Middle Aged
MH  - Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/blood/*diagnosis/*drug 
      therapy
MH  - Prospective Studies
MH  - Time Factors
MH  - Treatment Outcome
PMC - PMC6624149
EDAT- 2019/07/30 06:00
MHDA- 2020/09/25 06:00
PMCR- 2019/07/03
CRDT- 2019/07/30 06:00
PHST- 2019/03/07 00:00 [received]
PHST- 2019/05/16 00:00 [accepted]
PHST- 2019/07/30 06:00 [entrez]
PHST- 2019/07/30 06:00 [pubmed]
PHST- 2020/09/25 06:00 [medline]
PHST- 2019/07/03 00:00 [pmc-release]
AID - NEURIMMINFL2019020529 [pii]
AID - 10.1212/NXI.0000000000000590 [doi]
PST - epublish
SO  - Neurol Neuroimmunol Neuroinflamm. 2019 Jul 3;6(5):e590. doi: 
      10.1212/NXI.0000000000000590. eCollection 2019 Sep.