Objective: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune-inflammatory disease of the peripheral nervous system. Recently, various immunoglobulin G4 (IgG4) type auto-antibodies have been described in patients with CIDP which can effectively be removed by immunoadsorption (IA). Therefore, we prospectively evaluated the therapeutic effect of IA in 17 patients with progressive CIDP not responding to other treatment.
Methods: We prospectively evaluated the course of disease of 17 patients with CIDP who had insufficiently responded to steroids and/or IVIg previously and who received at least one cycle of IA. As clinical outcome parameter, we used a combined CIDP score of three validated scales comprising disability, motor score, and sensitivity. Seven patients received repeated treatments in regular intervals over a prolonged period up to 46 months.
Results: We observed a small, but significant improvement of the overall score after 2 weeks that mainly reflected an improvement of muscle strength. The median value of the combined CIDP score was 308.0 (266.0-374.5) points before IA and 330.0 (290.0-393.5) points 2 weeks after IA (p = 0.019). More importantly, all but one of seven progressive patients who received long-term immunoadsorption in regular intervals stabilized almost completely. Before IA, these patients lost 6.7 (3.0-13.1) points of combined CIDP score per month. During IA, they lost - 0.1 (0.0-0.8) points per month (p < 0.0001).
Interpretation: Our results suggest that IA might constitute a promising and well-tolerated therapeutic alternative in CIDP for short-term and long-term treatment. We showed that long-term treatment with IA in regular intervals can stabilize the course of disease at least in a subgroup of patients.
Keywords: CIDP; Chronic inflammatory demyelinating polyneuropathy; Immunoadsorption; Long-term effects.