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
N1 - Funding Information:
Medical writing support was provided by Dr. Barbara Boggetti of Trilogy Writing and Consulting GmbH, Frankfurt, Germany, funded by CSL Behring. Editorial support was provided by Meridian Health-Comms Ltd, funded by CSL Behring. Individual participant data will not be shared.
Publisher Copyright:
© 2019 CSL Behring. Journal of the Peripheral Nervous System published by Wiley Periodicals, Inc. on behalf of Peripheral Nerve Society.
PY - 2019/3
Y1 - 2019/3
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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UR - http://www.scopus.com/inward/citedby.url?scp=85062531892&partnerID=8YFLogxK
U2 - 10.1111/jns.12303
DO - 10.1111/jns.12303
M3 - Article
C2 - 30672067
AN - SCOPUS:85062531892
SN - 1085-9489
VL - 24
SP - 72
EP - 79
JO - Journal of the Peripheral Nervous System
JF - Journal of the Peripheral Nervous System
IS - 1
ER -