For Kids With Myasthenia Gravis, PLEX May Be Best Option

Mar 10, 2014

The first attempt to compare treatments for myasthenia gravis in patients who have not yet reached adulthood found that plasmapheresis provides significantly better results, whether alone or added to IVIG.

Liew WKM, Powell CA, Sloan SR, et al. Comparison of Plasmapheresis and Intravenous Immunoglobulin as Maintenance Therapies for Juvenile Myasthenia GravisJAMA Neurol (2014) Epub ahead of print. March 3, 2014. doi:10.1001/jamaneurol.2014.17

Plasmapheresis (PLEX) and intravenous immunoglobulin (IVIG) are both reasonable therapeutic options for juvenile myasthenia gravis (MG), but PLEX had a higher response rate in this retrospective analysis of 54 children and adolescents at Boston Children's Hospitals.

The effectiveness of PLEX and IVIG have been established in adult populations, but this was the first attempt to compare them in juvenile MG.

Over the course of 33 years, 27 patients with generalized juvenile MG received PLEX, IVIG, or both. All 7 patients treated with PLEX alone responded, five of the 10 treated with IVIG alone responded, and nine of 10 who received both responded; thus including PLEX produced a significantly better result.

Twenty-one patients had the ocular subtype, and 33 had the generalized subtype of juvenile MG.

Pyridostigmine was used in almost all children, except those with minimal ocular signs or symptoms, or spontaneous resolution. Patients who didn’t improve on pyridostigmine received immunomodulatory or immunosuppressive treatment with PLEX, IVIG, corticosteroids or azathioprine. Thymectomy was performed in 17 patients.

Pharmacological remission was achieved with pyridostigmine alone in six of 33 children with generalized juvenile MG and eight of 21 patients with ocular juvenile MG.

 

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