Long-term safety and efficacy of subcutaneous immunoglobulin IgPro20 in CIDP: PATH extension study

Ivo N. van Schaik*, Orell Mielke, Vera Bril, Nan van Geloven, Hans-Peter Hartung, Richard A. Lewis, Gen Sobue, John-Philip Lawo, Michaela Praus, Billie L. Durn, David R. Cornblath, Ingemar S. J. Merkies, A. Sabet, K. George, L. Roberts, R. Carne, S. Blum, R. Henderson, P. Van Damme, J. DemeestereS. Larue, C. D'Amour, Vera Bril, A. Breiner, P. Kunc, M. Michal, J. Sussova, K. Tomas, R. Talab, B. Michal, T. Toomsoo, Rubanovits, K. Gross-Paju, U. Sorro, M. Saarela, M. Auranen, J. Pouget, S. Attarian, G. Le Masson, A. Wielanek-Bachelet, C. Desnuelle, E. Delmont, P. Clavelou, D. Aufauvre, J. Schmidt, M. Mori, N. Visser, C. Faber, J. Hoeijmakers, PATH Study Grp

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

24 Citations (Web of Science)

Abstract

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.

Original languageEnglish
Article number590
Number of pages13
JournalNeurology: Neuroimmunology & Neuroinflammation
Volume6
Issue number5
DOIs
Publication statusPublished - Sep 2019

Keywords

  • INFLAMMATORY DEMYELINATING POLYNEUROPATHY
  • MUSCLE STRENGTH
  • MMN

Cite this