Abstract
The inflammatory neuropathies are disabling conditions with diverse immunological mechanisms. In some, a pathogenic role for immunoglobulin G (IgG)-class autoantibodies is increasingly appreciated, and immunoadsorption (IA) may therefore be a useful therapeutic option. We reviewed the use of and response to IA or plasma exchange (PLEx) in a cohort of 41 patients with nodal/paranodal antibodies identified from a total of 573 individuals with suspected inflammatory neuropathies during the course of routine diagnostic testing (PNAb cohort). 20 patients had been treated with PLEx and 4 with IA. Following a global but subjective evaluation by their treating clinicians, none of these patients were judged to have had a good response to either of these treatment modalities. Sequential serology of one PNAb+ case suggests prolonged suppression of antibody levels with frequent apheresis cycles or adjuvant therapies, may be required for effective treatment. We further retrospectively evaluated the serological status of 40 patients with either Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP), and a control group of 20 patients with clinically-isolated syndrome/multiple sclerosis (CIS/MS), who had all been treated with IgG-depleting IA (IA cohort). 32 of these patients (8/20 with CIDP, 13/20 with GBS, 11/20 with MS) were judged responsive to apheresis despite none of the serum samples from this cohort testing positive for IgG antibodies against glycolipids or nodal/paranodal cell-adhesion molecules. Although negative on antigen specific assays, three patients’ pre-treatment sera and eluates were reactive against different components of myelinating co-cultures. In summary, preliminary evidence suggests that GBS/CIDP patients without detectable IgG antibodies on routine diagnostic tests may nevertheless benefit from IA, and that an unbiased screening approach using myelinating co-cultures may assist in the detection of further autoantibodies which remain to be identified in such patients.
Highlights
The inflammatory neuropathies are a heterogeneous group of disorders in which peripheral nerve function and structure are disturbed by largely ill-defined immunological mechanisms [1]
Since August 2017, serum samples from a further 537 external patients with confirmed or suspected inflammatory neuropathies have been received for diagnostic nodal/paranodal antibody testing by the Oxford laboratory
The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) was subsequently revised to motor neuron disease; the diagnosis of an inflammatory neuropathy was retained at follow up in all other antibody positive cases
Summary
The inflammatory neuropathies are a heterogeneous group of disorders in which peripheral nerve function and structure are disturbed by largely ill-defined immunological mechanisms [1] They can broadly be divided into acute and chronic forms, typified by the umbrella terms Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP), respectively. Randomised controlled trials have demonstrated that therapeutic plasma exchange (PLEx) speeds up recovery from GBS [4], and provides at least a short-term improvement in disability in CIDP [5]. In both conditions there is evidence that intravenous immunoglobulin (IVIg) has similar efficacy [6,7]. There is some evidence that apheresis can improve recovery from multiple sclerosis relapses, and these approaches are often used after inadequate responses to corticosteroids [24,25]
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