Science News: Clinical Features and Treatment Response in Immune-Mediated Small Fiber Neuropathy with Trisulfated Heparin Disaccharide or Fibroblast Growth Factor Receptor 3 Antibodies

Submitted by: Betul Gundogdu, MD
Edited by: Milvia Y. Pleitez, MD

Zeidman LA, Kubicki K. Clinical features and treatment response in immune-mediated small fiber neuropathy with trisulfated heparin disaccharide or fibroblast growth factor receptor 3 antibodies. J Clin Neuromuscul Dis. 2021;22(4):192-199. doi:10.1097/CND.0000000000000355

Summary: The objective of the article was to describe clinical features and treatment responses in cryptogenic small fiber neuropathy (SFN) cases harboring novel antibodies to trisulfated heparin disaccharide (TS-HDS) and fibroblast growth factor receptor 3 (FGFR-3).
 
In this retrospective analysis, 40 cases of cryptogenic SFN with positive TS-HDS and FGFR-3 cases were identified. In this cohort, 95% were women and 55% had either TS-HDS or FGFR-3 antibodies (77% of these had TS-HDS). Of the seropositive group, 41% had a non-length dependent epidermal nerve fiber density on skin punch biopsy (OR = 1.80). In the seropositive group, 82% had neuropathic pain as their primary symptom (OR = 1.73). Also 32% of seropositive patients reported widespread pain (OR = 1.63). 63% of seropositive cases presented acutely (OR = 11.0). In the seropositive group, 23% had an initial erroneous diagnosis (OR = 1.47). Eight seropositive patients improved on intravenous immunoglobulin treatment, with a 42% reduction in pain scores (P = 0.02), a 44% reduction in the Utah Neuropathy Score, and improved epidermal nerve fiber density post-treatment.
 
As a conclusion, this study confirms that TS-HDS and FGFR-3 antibodies may be present in a high proportion of cryptogenic SFN cases with acute onset, non-length dependent pathology, and primary neuropathic and widespread pain. These cases may be responsive to immune treatment, especially with intravenous immunoglobulin.

Comments: This article is important as it demonstrates SFN with associated TS-HDS and FGFR-3 antibodies may respond to immunomodulatory treatment.

Article of Similar Interest: TS-HDS and FGFR3 antibodies in small fiber neuropathy and Dysautonomia. Trevino JA, Novak P. Muscle & Nerve. 2021 Jul;64(1):70-76. doi: 10.1002/mus.27245. Epub 2021 Apr 15.