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Little is known about long-term outcomes in patients with neuropathy and neurofascin-155 immunoglobulin G4 (NF155-IgG4) antibodies, and even less about the clinical significance of the presence of other NF155-IgG subtypes or NF155-IgM. To that end, researchers at one institution used an in-house flow cytometry-based assay to retrospectively identify 14 out of 214 (6.5%) patients with chronic inflammatory demyelinating neuropathy (CIDP), and 6 prospectively, who had NF155-IgG4 antibodies. Mean follow-up was 7 years.
As in prior NF155-IgG4 cohorts, common characteristics included lower extremity predominant distal weakness, pain, ataxia, and tremor. Most were refractory to intravenous immunoglobulin, and 90% required additional immunotherapy. …