IVIG Effective in Non-Length Dependent Skin Biopsies in Small Fiber Neuropathy with Plexin D1, TS-HDS, and FGFR-3 Antibodies (P9-11.007)

Document Type

Conference Proceeding

Publication Date

4-9-2024

Publication Title

Neurology

Keywords

autoantibody, fibroblast growth factor 3, heparin, immunoglobulin, plexin, adult, conference abstract, epidermis, female, headache, human, male, prevalence, questionnaire, randomized controlled trial, retrospective study, side effect, skin biopsy, small fiber neuropathy, therapy

Abstract

Objective: To demonstrate efficacy of Intravenous Immunoglobulin (IVIG) in pure small fiber neuropathy (SFN) patients who had trisulfated heparin disaccharide (TS-HDS), fibroblast growth factor-3 (FGFR-3), or Plexin D1 antibodies (seropositive). Background: SFN has an increasing prevalence and over 30% of cases may be immune-mediated. TS-HDS, FGFR-3, and Plexin D1 autoantibodies have been shown to be present in 44-55% of cryptogenic SFN cases, suggesting an immune-mechanism. Some reports have shown IVIG to be an effective treatment. Design/Methods: In a retrospective review, all pure SFN cases tested for the 3 antibodies from January 2021-May 2022 were tabulated, and cases who underwent IVIG treatment were separated and analyzed for changes in length-dependent (LD)-, non-length dependent (NLD)-, and composite-epidermal nerve fiber density (ENFD) on skin biopsy (composite is an average of all sites), as well as SFN-specific questionnaire and pain scores. Results: 92 patients with pure SFN had antibody testing (68% seropositive, 32% seronegative). 17 seropositive patients underwent IVIG treatment. Of these, 2 patients stopped treatment due to side effects (severe headaches or allergic reaction), and the remaining 15 completed at least 6 months of IVIG. Of these, 12 had a post-IVIG skin biopsy, and of these, 11 (92%) had a 55.1% mean improved composite ENFD (p=0.01). NLD-ENFD specimens improved by 42.3% (p=0.02) and LD-ENFD specimens improved by 99.7% (p=0.01). Composite ENFD in Plexin D1-SFN patients improved by 139% (p=0.04). Also, 14 patients had questionnaires pre-/post-IVIG and average pain decreased by 2.7 (p=0.002). Conclusions: IVIG shows disease-modifying effect in immune SFN with novel antibodies, especially Plexin D1-SFN, as well as significantly improved pain. NLD-ENFD should be examined as well as LD-ENFD to see this effect. Further randomized controlled trials looking at NLD- as well as LD-ENFD improvement, along with pain and SFN-specific questionnaires, are needed to confirm these findings.

Volume

102

Issue

17_supplement_1

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