Subcutaneous IgG for chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy and often responds to corticosteroids, plasmapheresis, and intravenous immunoglobulin (IVIg). 1 Dalakas MC Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011; 7: 507-511 Crossref PubMed Scopus (187) Google Scholar The only US Food and Drug Administration-approved, and the most commonly applied therapy, is IVIg, which, at 2 g/kg followed by 1 g/kg every 3–4 weeks, has been shown to prevent relapses and maintain stability in 75% of patients. 2 Hughes RA Donofrio P Bril V et al. for the ICE Study GroupIntravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo controlled trial. Lancet Neurol. 2008; 7: 136-144 Summary Full Text Full Text PDF PubMed Scopus (512) Google Scholar Subcutaneous immunoglobulin (SCIg), an alternative route of IgG administration, is approved for IgG replacement therapy in primary immunodeficiency, 3 Berger M Choices in IgG replacement therapy for primary immune deficiency diseases: subcutaneous IgG vs. intravenous IgG and selecting an optimal dose. Curr Opin Allergy Clin Immunol. 2011; 11: 532-538 Crossref PubMed Scopus (67) Google Scholar but whether or not it has an effect in balancing immune dysregulation in autoimmune diseases, as IVIg does, 4 Dalakas MC The use of intravenous immunoglobulin in the treatment of autoimmune neurological disorders: evidence-based indications and safety profile. Pharmacol Ther. 2004; 102: 177-193 Crossref PubMed Scopus (193) Google Scholar has not been formally tested. In The Lancet Neurology, Ivo van Schaik and colleagues 5 van Schaik IN Bril V van Geloven N et al. for the PATH study groupSubcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Neurol. 2017; (published online Nov 6.)http://dx.doi.org/10.1016/S1474-4422(17)30378-2 PubMed Google Scholar examined the efficacy of SCIg in CIDP and report that, in a 24 week period, 19 (33% [95% CI 22–46]) of 58 patients receiving high-dose SCIg and 22 (39% [27–52]) of 57 receiving low-dose SCIg relapsed or withdrew, compared with 36 (63% [50–74·5]) of 57 receiving placebo. 5 van Schaik IN Bril V van Geloven N et al. for the PATH study groupSubcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Neurol. 2017; (published online Nov 6.)http://dx.doi.org/10.1016/S1474-4422(17)30378-2 PubMed Google Scholar They conclude that SCIg is efficacious as maintenance therapy in IVIg-dependent patients. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trialThis study, which is to our knowledge, the largest trial of CIDP to date and the first to study two administrations of immunoglobulins and two doses, showed that both doses of SCIg IgPro20 were efficacious and well tolerated, suggesting that SCIg can be used as a maintenance treatment for CIDP. Full-Text PDF