Abstract
Objective The efficacy of maintenance intravenous immunoglobulin (IVIg) therapy has been established to prevent relapse in chronic inflammatory demyelinating polyneuropathy (CIDP). This prospective post-marketing surveillance study evaluated the treatment duration, efficacy, and safety of maintenance IVIg therapy in Japanese patients with CIDP. Methods Patients were registered between June 2017 and December 2018. After induction of IVIg therapy (0.4 g/kg/day for 5 consecutive days), patients received maintenance IVIg (1.0 g/kg every 3 weeks). The observation period was 18 months. Efficacy in preventing relapse was assessed using the Inflammatory Neuropathy Cause and Treatment (INCAT) disability score. Relapse was defined as a worsening of the INCAT score by ≥1 from baseline. Patients The efficacy population comprised 103 patients (80 with typical CIDP and 23 with CIDP variants). Results During the observation period, 86 (83%) patients were scheduled to continue maintenance IVIg therapy during the observation period, and the relapse rate was 24% (21/86). In the remaining 17 patients who showed continuous remission, maintenance IVIg therapy was stopped (mean, 136 days after the start), and the relapse rate was 24% (4/17). One serious adverse drug reaction of cardiac failure was reported. Conclusion In this post-marketing surveillance, most (83%) patients with CIDP were scheduled to continue maintenance IVIg for 18 months, with a relapse rate of 24%, indicating long-term efficacy. Maintenance IVIg therapy was rarely withdrawn, and the relapse rate after withdrawal was 24%. Further studies are required to determine the optimal maintenance IVIg dose and duration.