Background: Immune-mediated neuromuscular
conditions often cause significant disability and may require ongoing
immunomodulating therapies such as immunoglobulin (Ig). Ig use in several
neuromuscular conditions such as Chronic Inflammatory Demyelinating
Polyneuropathy (CIDP) is supported by robust evidence, however Ig is
increasingly used for other disorders. In British Columbia (BC), Ig use has
increased annually; last year, expenditure exceeded 51 million dollars, 35%
relating to neurological disease. Within the context of the pandemic, Ig
supply is at risk of shortages. Methods: A focused
literature review was conducted of CIDP, Guillain-Barré Syndrome (GBS),
Multifocal Motor Neuropathy (MMN), Myasthenia Gravis (MG), and other
neuromuscular conditions to compare BC Ig guidelines with international best
practices. Provincial recommendations for Ig use were updated accordingly.
Results: Evidence-based practices include acute
and chronic Ig use in CIDP and MMN, and acute or relapse-related treatment
in GBS and MG. Ig may be beneficial in other treatment-refractory
inflammatory disorders such as Lambert-Eaton Myasthenic Syndrome and
vasculitic neuropathy. Objective outcome measures can optimize patient care
and ensure appropriate resource utilization.
Conclusions: Updated BC guidelines emphasize
using established diagnostic criteria, objective outcome measures and
minimum effective Ig doses for neuromuscular conditions. Periodic literature
reviews on Ig use allow guidelines to remain evidence-based.