Maintenance intravenous immunoglobulin in chronic inflammatory demyelinating polyneuropathy: trends from an Australian tertiary centre

Abstract

Abstract

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is commonly treated with long-term intravenous immunoglobulin (IVIg). Individual dosing requirements and duration of IVIg differ among patients, leading to significant heterogeneity in IVIg utilisation. AIMS: We aimed to report on IVIg treatment trends in CIDP patients at our centre and sought to determine whether any clinical or demographic factors were predictive of a successful treatment wean. METHODS: This retrospective observational study included adults receiving maintenance IVIg for CIDP over a 5-year period across a hospital network in South Australia. Overall, IVIg maintenance requirements were described across the cohort. Patient characteristics, investigations and IVIg usage were recorded. These factors were compared between those who had successfully weaned IVIg and those who had not. RESULTS: . Mean cerebrospinal fluid (CSF) protein was 0.8 g/L. Mean maintenance IVIg was 0.6 g/kg every 4 weeks, with a mean duration of 64 months (range 3-152). Five out of six who had an IVIg wean successfully withdrew from IVIg completely. Exploratory analysis was limited by a small sample size; however, no significant association between clinical factors and successful IVIg wean were identified. CONCLUSION: Clinical stability was achieved for most patients on ≤0.6 g/kg/4 weeks of IVIg. Many individuals did not undergo an IVIg wean, highlighting the need for more proactive consideration of dose reduction or treatment cessation trials.