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