TS-HDS autoantibody: clinical characterization and utility from real-world tertiary care center experience.

Abstract

OBJECTIVES:

To evaluate clinical utility of trisulfated-heparin disaccharide (TS-HDS) IgM testing from real-world tertiary care center experience.

METHODS:

Medical records of patients with positive TS-HDS antibodies who were evaluated at Mayo Clinic from 2009 to 2022 were reviewed.

RESULTS:

Seventy-seven patients (50 females) had positive TS-HDS antibody. Median age was 48 (9-77) years. Median titer was 25,000 (range 11,000-350,000). Twenty-six patients (34%) did not have objective evidence of peripheral neuropathy. Nine patients (12%) had other known causes of neuropathy. Among the remaining 42 patients, half presented with subacute progressive course; the other half had chronic indolent course. Most common phenotypes were length-dependent peripheral neuropathy (n = 20, 48%), length-dependent small-fiber neuropathy (n = 11, 26%), and non-length-dependent small-fiber neuropathy (n = 7, 17%). Nerve biopsies showed epineurial inflammatory cell collections in 2 but no interstitial abnormalities in the remaining 7. The majority of intraepidermal nerve fiber densities (7/10), thermoregulatory sweat tests (12/21) and autonomic reflex screens (27/49) were normal. Post-immunotherapy improvement in mRS/INCAT disability score/pain was only seen in 13/42 (31%) TS-HDS IgM positive patients. Patients presenting with sensory ganglionopathy, non-length dependent small-fiber neuropathy, or subacute progressive neuropathy with and without TS-HDS antibody responded similarly to immunotherapy (40% vs 80%, p = 0.30).

DISCUSSION:

TS-HDS IgM has limited phenotypic or disease specificity; it was found to be positive among patients with various neuropathy phenotypes as well as patients without objective evidence of neuropathy. Clinical improvement with immunotherapy, although was observed in a small proportion of TS-HDS IgM seropositive patients, was not more frequent when compared to seronegative patients with similar presentations.

• Authors: Pitcha Chompoopong, Mohamed Rezk, Igal Mirman, Sarah E Berini, P James B Dyck, Michelle Mauermann, Kamal Shouman, Christopher Klein, John R Mills, Divyanshu Dubey
• Journal: Journal of neurology (J Neurol) Vol. 270 Issue 9 Pg. 4523-4528 (Sep 2023) ISSN: 1432-1459 [Electronic] Germany
• PMID: 37294321 (Publication Type: Journal Article)
• Copyright: © 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.

Chemical References

• heparin disaccharide
• Autoantibodies
• Immunoglobulin M

Topics

• Female
• Humans
• Middle Aged
• Autoantibodies
• Tertiary Care Centers
• Peripheral Nervous System Diseases (diagnosis, pathology)
• Small Fiber Neuropathy
• Immunoglobulin M