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Abstract Details

Small Vessel Vasculitis in Small Fiber Neuropathy with TS-HDS, FGFR-3, or Plexin D1 antibodies
Neuromuscular and Clinical Neurophysiology (EMG)
P14 - Poster Session 14 (11:45 AM-12:45 PM)
10-007

SFN is increasingly prevalent, and a high proportion of previously idiopathic cases may have TS-HDS, FGFR-3, or Plexin-D1 autoantibodies (seropositive). Previously immunoreactivity of TS-HDS, FGFR-3, and Plexin D1 has been demonstrated on human nerves or animal sensory neurons. No study has shown inflammation on seropositive skin biopsy specimens.

To describe and analyze small fiber neuropathy (SFN) cases with autoantibodies and punch biopsy specimens with inflammation. 

A retrospective analysis of all punch biopsies in seropositive cases revealed 83 patients, of which 5 (6%) had superficial lymphocytic perivascular inflammation.  Demographics and clinical features were analyzed.

Of these 5, 3 were female (60%) and 2 were male (40%). The average age of onset was 39.4 (16-57). Five patients (100%) had pain, 4 had burning (80%), 4 had numbness (80%), 5 had paresthesias (100%), and 2 had dysautonomia (40%). One patient (20%) had facial/head involvement, 2 (40%) had truncal, 4 (80%) had arm, and 5 (100%) had leg involvement. Two patients had TS-HDS and 1 patient had TS-HDS and Plexin D1 antibodies, 1 had FGFR-3 and 1 had FGFR-3 and Plexin D1 antibodies. The average Utah Early Neuropathy Scale score was 3, pain score 4.8/10, SFN-RODS 47.6, SFN-SIQ 12.6 and SFN-SL 31.4. Three patients (60%) had non-length dependent (NLD) inflammation present, whereas 1 (43%) different patient had NLD-epidermal nerve fiber density.2 patients (40%) had CD3-immunostain showing a predominance of T-cells in the infiltrate. No patients received immunomodulatory treatment yet.

Inflammation was seen in a small portion of seropositive biopsies, but suggests an immune mechanism at the ganglion level, and provides further proof of autoimmunity in seropositive-SFN. These patients often have moderate to severe SFN symptoms and pain, exam findings, and disability. Further studies may be indicated to assess whether biopsy-inflammation is a marker for immunomodulatory treatment responsiveness.

Authors/Disclosures
Lawrence A. Zeidman, MD, FAAN (Henry Ford Health)
PRESENTER
Dr. Zeidman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Pfizer. Dr. Zeidman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen. Dr. Zeidman has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Grifols. The institution of Dr. Zeidman has received research support from Octapharma. Dr. Zeidman has received publishing royalties from a publication relating to health care.