Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: the ICE trial

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a potentially disabling autoimmune disease causing progressive or relapsing-remitting weakness with or without sensory loss. Previous small trials demonstrated short-term benefit from intravenous immunoglobulin (IVIg), and international guidelines recommend IVIg as an option. However, evidence had been insufficient to persuade authorities to approve IVIg for use in CIDP. This article aims to review the Immune Globulin Intravenous CIDP Efficacy (ICE) trial, which was a randomized, double-blind, placebo-controlled, response-conditional crossover trial of Gamunex (intravenous immunoglobulin, 10% caprylate/chromatography purified). With 117 participants, it is the largest treatment trial ever conducted in CIDP. The results showed unequivocal short- and long-term benefit from IVIg in confirmation of previous reports. The trial also showed for the first time that continued IVIg infusion 1 g/kg every 3 weeks protected participants from relapse. Adverse events were mostly mild and serious adverse events were not more common with IVIg than with placebo. The results persuaded the US FDA and Health Canada to approve Gamunex for use in CIDP.

Sub-acute Demyelinating Polyradiculoneuropathy as an Initial Symptom of Peripheral T Cell Lymphoma, Not Otherwise Specified (PTCL-NOS)

“…In addition, although we could not detect any autoantibody related to PNS in our patient, an unidentified autoantibody might cause peripheral neuropathy with a similar mechanism observed in PNS. In agreement with our speculation, his neurological symptoms and signs were slightly improved by steroid therapy and IVIG, both of which are established treatments for CIDP and other immune-mediated neuropathies (15)(16)(17). Together, these results suggest the involvement of an immunemediated process in his neuropathy.…”

Autologous haematopoietic stem cell transplantation: a viable treatment option for CIDP

“…11. Disability prior to and following AHSCT was measured using the 0–10 point INCAT scale3 20 as well as the 0–6 point modified Rankin (mRANKIN) scale 21. In patients examined prior to the introduction of the INCAT score in 2001, the INCAT scores were recreated retrospectively using chart data obtained from treating neurologists, physiotherapists and occupational therapists (table 1).…”

“…First-line treatments in CIDP consist of intravenous high-dose immunoglobulin (IVIg), corticosteroids and plasma exchange (PE) 2 3. Approximately 70% of patients with CIDP respond adequately to one of the first-line treatments, and a further 10% upon switching of therapies 4.…”

Immunoglobulins for primary or secondary immunodeficiency or for immunomodulation in neurological autoimmune diseases: insights from the prospective SIGNS registry

“…However, despite some small early trials that have shown some efficacy of IVIG in relapsing-remitting MS, this could not be reproduced in a recent placebo-controlled trial (Cohen 2008;Fazekas et al 2008). There are stronger data available for CIDP, where in addition to some small studies the double-blind, randomized ICE trial (Hughes et al 2008) in 117 patients showed superiority of IG over placebo (Hughes 2009). Also for multifocal motor neuropathy (MMN) characterized by asymmetric weakness of limbs and the electrophysiological finding of conduction block in motor nerves, selected IG preparations have been approved for treatment on the basis of randomized studies (Dalakas 2004;Eftimov et al 2009;Harbo et al 2009).…”

“…Non-modified human IG are standard of care for replacement therapy with primary (inherited) immunodeficiencies (PID), and secondary immunodeficiencies (SID) due to multiple myeloma, chronic lymphocytic leukemia (CLL) or other malignancies. IG have also effectively been used as immunomodulatory therapy in neurological autoimmune diseases (AID) (Hughes et al 2009;Stangel and Gold 2010). Clinical studies have shown efficacy of high dose intravenous IG (IVIG) in Guillain Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), or multifocal motoric neuropathy (MMN) and provided the basis for regulatory approval of some preparations in these indications (Elovaara et al 2008;Stangel and Gold 2010).…”