Antibodies to peripheral nerve myelin proteins in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disorder of the peripheral nervous system with a probable auto-immune pathogenesis. The nature of the responsible autoantigens is unclear in most patients. We used the Western immunoblot technique to seek antibodies to peripheral nerve protein antigens. Sera from eight of 32 (25%) CIDP patients, 12 of 37 (32%) Guillain-Barré syndrome (GBS) patients, zero of 30 (0%) chronic idiopathic axonal polyneuropathy patients and two of 39 (5%) healthy control subjects contained anti-peripheral nerve protein antibodies. The frequency of such antibodies was significantly greater in both CIDP (p = 0.04) and GBS (p = 0.003) patients than in normal control subjects. For CIDP patients, there were non-significant trends for antibodies to be more common in females and in those who responded to treatment with either intravenous immunoglobulin or plasma exchange. The commonest antibodies were directed against a band at 28 kDa, resembling that labelled by a monoclonal antibody against myelin protein zero (P0). Six CIDP and seven GBS patients' sera reacted with this band. These results support the view that antibodies to myelin proteins, and especially P0, are present in the serum of some patients with CIDP and GBS.

Guillain–Barré syndrome: update on immunobiology and treatment

“…The predominant pathology of the EAN shows infiltration of T cells and macrophages and demyelination, similar to the histopathology of AIDP [7,68]. Indeed, sera from patients with AIDP often show antibodies to various peripheral nerve myelin, but it is not clear how the antibodies are involved with thr pathophysiology of AIDP [69][70][71]. The mechanism of conduction failure and demyelination in AIDP has also not been well understood.…”

Humoral and cellular immune responses to myelin protein peptides in chronic inflammatory demyelinating polyradiculoneuropathy

“…Myelin protein zero (P0), myelin protein 2 (P2) and peripheral myelin protein 22 (PMP22) are the principal candidates as target antigens because they can each induce experimental autoimmune neuritis (EAN) 2 8. Antibodies to myelin proteins have been reported with variable frequencies in CIDP in previous studies but not consistently 4 9 – 16. Cellular reactivity to myelin proteins has only been studied infrequently 5 17 – 20.…”

Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

“…15 Allen and colleagues also found antibodies to P0-like bands by immunoblot in eight of 32 patients with CIDP and none in 30 normal controls. 16 However, Sanvito et al did not find a significant difference in the proportion of subjects with IgG anti-P0 peptides in patients with CIDP compared with controls, although individual subjects with CIDP had high titers. In this study there was a higher proportion of untreated CIDP patients compared with healthy controls (4/18 vs 0/32) with IgG antibodies to P2.…”