![]() This is advantageous in reducing complications, such as those due to the unwanted removal of coagulation factors, but may also lead to a loss of therapeutic effect if this depends on the removal of pro-inflammatory cytokines, or other pathogenically-relevant molecules, rather than immunoglobulins. Replacement fluid is not required in IA, and the range of circulating factors removed is more limited. ![]() ![]() ![]() The former removes a broad range of circulating molecules and requires the use of replacement fluid, typically fresh frozen plasma, or albumin. There are of course substantial differences between PLEx and IA. There is also some evidence that apheresis can improve recovery from multiple sclerosis relapses, and these approaches are often used after inadequate responses to corticosteroids. However, high-quality evidence demonstrating the efficacy of IA in the inflammatory neuropathies is lacking. A retrospective Japanese report of IA in GBS found that patients who received IA within 6 days of onset of their neuropathy had a more rapid improvement in disability compared to those who received supportive care alone, whereas patients who received IA later than this in their disease course did not. A number of retrospective case series and case reports have favourably evaluated immunoadsorption in both GBS and CIDP. Two further reports described the crossover from PLEx to IA in CIDP, in a single patient each, reaching opposite conclusions about which was more efficacious. The trial comparing IA (using protein A) with IVIg had a high drop-out rate and was excluded from the relevant Cochrane review due to a high risk of bias. Response rates to IA (6/9 using tryptophan-based columns and 4/5 using protein A ) were not significantly different to their respective comparators. Two small, randomised studies have compared immunoadsorption (IA) with PLEx or IVIg in CIDP. In both conditions there is evidence that intravenous immunoglobulin (IVIg) has similar efficacy. Randomised controlled trials have demonstrated that therapeutic plasma exchange (PLEx) speeds up recovery from GBS, and provides at least a short-term improvement in disability in CIDP. 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. Although negative on antigen specific assays, three patients’ pre-treatment sera and eluates were reactive against different components of myelinating co-cultures. 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. 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). 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. 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. 20 patients had been treated with PLEx and 4 with IA. 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). In some, a pathogenic role for immunoglobulin G (IgG)-class autoantibodies is increasingly appreciated, and immunoadsorption (IA) may therefore be a useful therapeutic option. The inflammatory neuropathies are disabling conditions with diverse immunological mechanisms.
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