PMID- 36005471
OWN - NLM
STAT- MEDLINE
DCOM- 20220826
LR  - 20220826
IS  - 1537-1611 (Electronic)
IS  - 1522-0443 (Linking)
VI  - 24
IP  - 1
DP  - 2022 Sep 1
TI  - Immune-Mediated Small Fiber Neuropathy With Trisulfated Heparin Disaccharide, 
      Fibroblast Growth Factor Receptor 3, or Plexin D1 Antibodies: Presentation and 
      Treatment With Intravenous Immunoglobulin.
PG  - 26-37
LID - 10.1097/CND.0000000000000423 [doi]
AB  - OBJECTIVES: Up to 50% of small fiber neuropathy (SFN) cases are idiopathic, but 
      novel antibodies to Trisulfated Heparin Disaccharide (TS-HDS) and fibroblast 
      growth factor receptor 3 (FGFR-3) have been implicated in half of these cases; 
      the role of anti-Plexin D1 is less clear. We aimed to clarify presentation and 
      management of these patients. METHODS: An 18-month retrospective analysis 
      revealed 54 cases of cryptogenic SFN who had testing for the 3 autoantibodies. 
      Demographics, clinical features, epidermal nerve fiber density, and Quantitative 
      Sudomotor Axon Reflex Test results were analyzed. Intravenous immunoglobulin 
      (IVIG) treatment response was assessed. RESULTS: In total, 44.4% of patients had 
      antibodies (62.5% TS-HDS, 29.2% FGFR-3, and 20.8% Plexin D1). Male patients were 
      more likely to be FGFR-3 positive (P = 0.014). Facial involvement was more common 
      in seropositive patients (P = 0.034), and patients with a higher Utah Early 
      Neuropathy Scale score had a higher TS-HDS titer (P = 0.0469), but other clinical 
      features were not significantly different. Seropositive patients trended toward a 
      higher SFN screening list score (P = 0.16), abnormal Quantitative Sudomotor Axon 
      Reflex Test (P = 0.052), and prior erroneous diagnosis (P = 0.19). In patients 
      who completed IVIG, examinations and questionnaires improved and mean epidermal 
      nerve fiber density increased by 297%. CONCLUSIONS: TS-HDS, FGFR-3, and Plexin D1 
      antibodies are present in a high proportion of cryptogenic SFN cases with more 
      facial involvement, and greater disease severity is associated with higher 
      antibody titers. They are often misdiagnosed but may respond subjectively and 
      objectively to IVIG.
CI  - Copyright (c) 2022 Wolters Kluwer Health, Inc. All rights reserved.
FAU - Zeidman, Lawrence A
AU  - Zeidman LA
AUID- ORCID: 0000-0001-7268-7057
AD  - Arlington Heights, IL; Stritch School of Medicine, Loyola University Chicago, 
      Maywood, IL; Department of Neurology, University of Southern California, Los 
      Angeles, CA; and Department of Neurology, Barrow Neurological Institute, Phoenix, 
      AZ.
FAU - Saini, Pravesh
AU  - Saini P
FAU - Mai, Peter
AU  - Mai P
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Clin Neuromuscul Dis
JT  - Journal of clinical neuromuscular disease
JID - 100887391
RN  - 0 (Disaccharides)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (heparin disaccharide)
RN  - 9005-49-6 (Heparin)
RN  - EC 2.7.10.1 (Receptor, Fibroblast Growth Factor, Type 3)
SB  - IM
MH  - Disaccharides
MH  - Heparin/analogs & derivatives
MH  - Humans
MH  - Immunoglobulins, Intravenous/therapeutic use
MH  - Male
MH  - Receptor, Fibroblast Growth Factor, Type 3
MH  - Retrospective Studies
MH  - *Small Fiber Neuropathy/diagnosis/drug therapy
COIS- L. A. Zeidman has received honoraria for participating on scientific advisory 
      boards for Akcea Therapeutics and Takeda, for being on the CIDP speaker's bureau 
      for Grifols, has received fees for expert medicolegal consulting, and has 
      received royalties from Oxford University Press. The remaining authors report no 
      conflicts of interest.
EDAT- 2022/08/26 06:00
MHDA- 2022/08/27 06:00
CRDT- 2022/08/25 08:13
PHST- 2022/08/25 08:13 [entrez]
PHST- 2022/08/26 06:00 [pubmed]
PHST- 2022/08/27 06:00 [medline]
AID - 00131402-202209000-00004 [pii]
AID - 10.1097/CND.0000000000000423 [doi]
PST - ppublish
SO  - J Clin Neuromuscul Dis. 2022 Sep 1;24(1):26-37. doi: 
      10.1097/CND.0000000000000423.