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

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

Twenty-two patients were included, with 82% meeting European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) criteria for definite CIDP. Seventeen had typical CIDP, with a mean age of 58 and a male-to-female ratio of 2:1. Average body mass index was 30 kg/m2. 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.