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Objective: To assess the efficacy and safety of intraveinous immunoglobulin (IV Ig) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) at 4, 7 and 12months., Methods: A national multicenter retrospective study was conducted by LFB Biotehcnologies in patients with CIDP who had received at least one cycle of a 5% polyvalent IV Ig, Tegeline(®), from LFB biomédicaments between 1995 and 2004. The primary endpoint was the efficacy of IV Ig at 4 months, which was defined as the responder rate based on the modified Rankin scale. Several secondary endpoints were assessed: safety and efficacy (i.e., responders according to the investigators' overall assessment of the patients' status) at 4, 7 and 12 months. The analysis was performed at 7 months only (due to missing data for 12 months and few patients)., Results: A total of 26 patients were included who had received between 1 and 6 cycles of IV Ig (mean 3±2) with a median follow-up of 9.9 months. The responder rate at 4 months based on the modified Rankin scale was 52% (95% CI 0.313-0.722), whereas the responder rate with placebo reported in the literature (meta-analysis including results from van Schaik and an ICE study) is 18% (P<0.001). Responder patients at 4 months were still responders at 7 months. The overall safety of IV Ig was good, with adverse events of mild to moderate severity, which resolved without sequelae and were expected adverse events of IV Ig., Conclusion: This retrospective study confirmed both the efficacy of IV Ig at 4 months in the treatment of chronic inflammatory demyelinating polyneuropathy and the favorable safety profile of the product., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Introduction: Primary Immunodeficiencies (PIDs) represent a heterogeneous group of rare diseases characterized by increased susceptibility to infections, often accompanied by a diverse immuno-pathological manifestations (autoimmunity, inflammation, benign or malignant lymphoproliferative disorders). The precise prevalence of PIDs in France is not known but is estimated to represent approximately 5 000 patients., Methods: To better understand the situation of PID in France and gain an insight as to the care of these patients, we conducted a national survey by sending a questionnaire to physicians potentially involved in the care of PID patients., Results: The majority of physicians follow only a few PID patients but the diagnostic and therapeutic attitudes are generally satisfactory., Discussion: These results underscore the need to coordinate the care of PID patients in France as part of national networks. The approach adopted by the CEREDIH (French reference center for hereditary immune deficiencies) and DEF-I french national cohort identifying patients with DIP, will optimize the management of PID by defining diagnostic and therapeutic guidelines. In addition, these networks will provide valuable data regarding the incidence of PID and their complications., (Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.)