Immune responses to myelin proteins in Guillain-Barré syndrome

Abstract

Background: Potential target autoantigens in the demyelinating form of Guillain-Barré syndrome (GBS) include the myelin proteins PMP22, P0 and P2.

Methods: We investigated immunoreactivity to P0, P2 and PMP22 proteins in 37 patients with GBS and 32 healthy controls.

Results: Antibodies to PMP22 or P0 peptides were detected at presentation in only 5 out of 37 patients. In ELISPOT assays, blood mononuclear cells from 15 out of 24 patients with GBS, but none of the control subjects, produced interleukin-10 (IL-10) in response to peptides from proteins P0, P2 or PMP22 (p = 0.0003). The cells from only two patients produced interferon-gamma (IFN gamma). The cells from 11 patients with GBS had increased IL-10 responses to peptides representing sequences from the extracellular domains of PMP22 before intravenous immunoglobulin (IVIg) treatment (p = 0.006). The cells from 11 patients with GBS, including 7 who responded to the extracellular domains of PMP22, had increased IL-10 responses to the intracellular domain of P0 before (p = 0.005) and those from 9 patients after they had been treated with IVIg (p = 0.01).

Conclusions: Antibodies to P0 and PMP22 protein peptides do occur in GBS but are uncommon. Circulating mononuclear cell IFN gamma responses to P0, P2 and PMP22 myelin protein peptides are rare, but IL-10 responses occur significantly more often than in normal subjects. They might be part of a harmful pathogenetic process or represent a regulatory response.