Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy

Results from the pre-randomization phase of the Polyneuropathy And Treatment with Hizentra study

on behalf of the PATH study group

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilization with IVIG in patients who worsen after IVIG withdrawal. In the Polyneuropathy And Treatment with Hizentra (PATH) study, the pre-randomization period included sudden stopping of IVIG followed by 12 weeks of observation. Those deteriorating were then restabilized with IVIG. Of 245 subjects who stopped IVIG, 28 did not show signs of clinical deterioration within 12 weeks. Two hundred and seven received IVIG restabilization with an induction dose of 2 g/kg bodyweight (bw) IgPro10 (Privigen, CSL Behring, King of Prussia, Pennsylvania) and maintenance doses of 1 g/kg bw every 3 weeks for up to 13 weeks. Signs of clinical improvement were seen in almost all (n = 188; 91%) subjects. During IVIG restabilization, 35 subjects either did not show CIDP stability (n = 21, analyzed as n = 22 as an additional subject was randomized in error) or withdrew for other reasons (n = 14). Of the 22 subjects who did not achieve clinical stability, follow-up information in 16 subjects after an additional 4 weeks was obtained. Nine subjects were reported to have improved, leaving a maximum of 27 subjects (13%) who either showed no signs of clinical improvement during the restabilization phase and 4 weeks post-study or withdrew for other reasons. In conclusion, sudden IVIG withdrawal was effective in detecting ongoing immunoglobulin G dependency with a small risk for subjects not returning to their baseline 17 weeks after withdrawal.

Original languageEnglish (US)
Pages (from-to)72-79
Number of pages8
JournalJournal of the Peripheral Nervous System
Volume24
Issue number1
DOIs
StatePublished - Mar 1 2019

Fingerprint

Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Polyneuropathies
Intravenous Immunoglobulins
Random Allocation
Therapeutics
Prussia
Hizentra
Immunoglobulin G
Observation

Keywords

  • Privigen
  • chronic inflammatory demyelinating polyneuropathy (CIDP)
  • inflammatory neuropathy cause and treatment (INCAT)
  • intravenous immunoglobulin (IVIG)
  • polyneuropathy and treatment with Hizentra (PATH)

Cite this

@article{a1b8ba121e90404d965810e9c88032ad,
title = "Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy: Results from the pre-randomization phase of the Polyneuropathy And Treatment with Hizentra study",
abstract = "In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilization with IVIG in patients who worsen after IVIG withdrawal. In the Polyneuropathy And Treatment with Hizentra (PATH) study, the pre-randomization period included sudden stopping of IVIG followed by 12 weeks of observation. Those deteriorating were then restabilized with IVIG. Of 245 subjects who stopped IVIG, 28 did not show signs of clinical deterioration within 12 weeks. Two hundred and seven received IVIG restabilization with an induction dose of 2 g/kg bodyweight (bw) IgPro10 (Privigen, CSL Behring, King of Prussia, Pennsylvania) and maintenance doses of 1 g/kg bw every 3 weeks for up to 13 weeks. Signs of clinical improvement were seen in almost all (n = 188; 91{\%}) subjects. During IVIG restabilization, 35 subjects either did not show CIDP stability (n = 21, analyzed as n = 22 as an additional subject was randomized in error) or withdrew for other reasons (n = 14). Of the 22 subjects who did not achieve clinical stability, follow-up information in 16 subjects after an additional 4 weeks was obtained. Nine subjects were reported to have improved, leaving a maximum of 27 subjects (13{\%}) who either showed no signs of clinical improvement during the restabilization phase and 4 weeks post-study or withdrew for other reasons. In conclusion, sudden IVIG withdrawal was effective in detecting ongoing immunoglobulin G dependency with a small risk for subjects not returning to their baseline 17 weeks after withdrawal.",
keywords = "Privigen, chronic inflammatory demyelinating polyneuropathy (CIDP), inflammatory neuropathy cause and treatment (INCAT), intravenous immunoglobulin (IVIG), polyneuropathy and treatment with Hizentra (PATH)",
author = "{on behalf of the PATH study group} and Orell Mielke and Vera Bril and Cornblath, {David R.} and Lawo, {John Philip} and {van Geloven}, Nan and Hartung, {Hans Peter} and Lewis, {Richard A.} and Merkies, {Ingemar S.J.} and Gen Sobue and Billie Durn and Amgad Shebl and {van Schaik}, {Ivo N.} and A. Sabet and K. George and L. Roberts and R. Carne and S. Blum and R. Henderson and {Van Damme}, P. and J. Demeestere and S. Larue and C. D'Amour and P. Kunc and M. Valis and J. Sussova and T. Kalous and R. Talab and M. Bednar and T. Toomsoo and I. Rubanovits and K. Gross-Paju and U. Sorro and M. Saarela and M. Auranen and J. Pouget and S. Attarian and {Le Masson}, G. and A. Wielanek-Bachelet and C. Desnuelle and E. Delmont and P. Clavelou and D. Aufauvre and J. Schmidt and J. Zschuentzsch and C. Sommer and D. Kramer and O. Hoffmann and C. Goerlitz and Allen, {Jeffrey A} and David Walk",
year = "2019",
month = "3",
day = "1",
doi = "10.1111/jns.12303",
language = "English (US)",
volume = "24",
pages = "72--79",
journal = "Journal of the Peripheral Nervous System",
issn = "1085-9489",
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number = "1",

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TY - JOUR

T1 - Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy

T2 - Results from the pre-randomization phase of the Polyneuropathy And Treatment with Hizentra study

AU - on behalf of the PATH study group

AU - Mielke, Orell

AU - Bril, Vera

AU - Cornblath, David R.

AU - Lawo, John Philip

AU - van Geloven, Nan

AU - Hartung, Hans Peter

AU - Lewis, Richard A.

AU - Merkies, Ingemar S.J.

AU - Sobue, Gen

AU - Durn, Billie

AU - Shebl, Amgad

AU - van Schaik, Ivo N.

AU - Sabet, A.

AU - George, K.

AU - Roberts, L.

AU - Carne, R.

AU - Blum, S.

AU - Henderson, R.

AU - Van Damme, P.

AU - Demeestere, J.

AU - Larue, S.

AU - D'Amour, C.

AU - Kunc, P.

AU - Valis, M.

AU - Sussova, J.

AU - Kalous, T.

AU - Talab, R.

AU - Bednar, M.

AU - Toomsoo, T.

AU - Rubanovits, I.

AU - Gross-Paju, K.

AU - Sorro, U.

AU - Saarela, M.

AU - Auranen, M.

AU - Pouget, J.

AU - Attarian, S.

AU - Le Masson, G.

AU - Wielanek-Bachelet, A.

AU - Desnuelle, C.

AU - Delmont, E.

AU - Clavelou, P.

AU - Aufauvre, D.

AU - Schmidt, J.

AU - Zschuentzsch, J.

AU - Sommer, C.

AU - Kramer, D.

AU - Hoffmann, O.

AU - Goerlitz, C.

AU - Allen, Jeffrey A

AU - Walk, David

PY - 2019/3/1

Y1 - 2019/3/1

N2 - In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilization with IVIG in patients who worsen after IVIG withdrawal. In the Polyneuropathy And Treatment with Hizentra (PATH) study, the pre-randomization period included sudden stopping of IVIG followed by 12 weeks of observation. Those deteriorating were then restabilized with IVIG. Of 245 subjects who stopped IVIG, 28 did not show signs of clinical deterioration within 12 weeks. Two hundred and seven received IVIG restabilization with an induction dose of 2 g/kg bodyweight (bw) IgPro10 (Privigen, CSL Behring, King of Prussia, Pennsylvania) and maintenance doses of 1 g/kg bw every 3 weeks for up to 13 weeks. Signs of clinical improvement were seen in almost all (n = 188; 91%) subjects. During IVIG restabilization, 35 subjects either did not show CIDP stability (n = 21, analyzed as n = 22 as an additional subject was randomized in error) or withdrew for other reasons (n = 14). Of the 22 subjects who did not achieve clinical stability, follow-up information in 16 subjects after an additional 4 weeks was obtained. Nine subjects were reported to have improved, leaving a maximum of 27 subjects (13%) who either showed no signs of clinical improvement during the restabilization phase and 4 weeks post-study or withdrew for other reasons. In conclusion, sudden IVIG withdrawal was effective in detecting ongoing immunoglobulin G dependency with a small risk for subjects not returning to their baseline 17 weeks after withdrawal.

AB - In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilization with IVIG in patients who worsen after IVIG withdrawal. In the Polyneuropathy And Treatment with Hizentra (PATH) study, the pre-randomization period included sudden stopping of IVIG followed by 12 weeks of observation. Those deteriorating were then restabilized with IVIG. Of 245 subjects who stopped IVIG, 28 did not show signs of clinical deterioration within 12 weeks. Two hundred and seven received IVIG restabilization with an induction dose of 2 g/kg bodyweight (bw) IgPro10 (Privigen, CSL Behring, King of Prussia, Pennsylvania) and maintenance doses of 1 g/kg bw every 3 weeks for up to 13 weeks. Signs of clinical improvement were seen in almost all (n = 188; 91%) subjects. During IVIG restabilization, 35 subjects either did not show CIDP stability (n = 21, analyzed as n = 22 as an additional subject was randomized in error) or withdrew for other reasons (n = 14). Of the 22 subjects who did not achieve clinical stability, follow-up information in 16 subjects after an additional 4 weeks was obtained. Nine subjects were reported to have improved, leaving a maximum of 27 subjects (13%) who either showed no signs of clinical improvement during the restabilization phase and 4 weeks post-study or withdrew for other reasons. In conclusion, sudden IVIG withdrawal was effective in detecting ongoing immunoglobulin G dependency with a small risk for subjects not returning to their baseline 17 weeks after withdrawal.

KW - Privigen

KW - chronic inflammatory demyelinating polyneuropathy (CIDP)

KW - inflammatory neuropathy cause and treatment (INCAT)

KW - intravenous immunoglobulin (IVIG)

KW - polyneuropathy and treatment with Hizentra (PATH)

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U2 - 10.1111/jns.12303

DO - 10.1111/jns.12303

M3 - Article

VL - 24

SP - 72

EP - 79

JO - Journal of the Peripheral Nervous System

JF - Journal of the Peripheral Nervous System

SN - 1085-9489

IS - 1

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