Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Muscle Nerve. 2021 Jul;64(1):70-76. doi: 10.1002/mus.27245. Epub 2021 Apr 15.
The specificity of trisulfated heparin disaccharide/fibroblast growth factor receptor 3 (TS-HDS/FGFR3) antibodies in the diagnosis of autoimmune small fiber neuropathy (SFN) is unclear.
This was a retrospective study of patients evaluated for SFN and dysautonomia in the Brigham and Women's Faulkner Hospital Autonomic Laboratory in 2019-2020. Associations were assessed between TS-HDS/FGFR3 antibodies and SFN markers, including epidermal nerve fiber density (ENFD), sweat gland nerve fiber density (SGNFD), and autonomic dysfunction assessed by Valsalva maneuver, deep breathing, sudomotor, and tilt testing.
Of 322 patients; 28% had elevated anti-TS-HDS, 17% had elevated anti-FGFR3, 96% had autonomic dysfunction, 71% had abnormal ENFD, and 49% had abnormal SGNFD. TS-HDS/FGFR3 antibodies were present in patients with autonomic dysfunction irrespective of whether they had normal or abnormal skin biopsies unless ENFD/SGNFD were combined for anti-FGFR3 seropositivity.
TS-HDS/FGFR3 antibodies are present in patients with evidence of autonomic dysfunction. Further studies are needed to document the clinical value of these antibodies in assessment of immune mediated dysautonomia.
三硫酸化肝素二糖/成纤维细胞生长因子受体 3（TS-HDS/FGFR3）抗体在自身免疫性小纤维神经病（SFN）诊断中的特异性尚不清楚。
这是 2019-2020 年在布莱根妇女医院福克纳医院自主实验室评估 SFN 和自主神经功能障碍患者的回顾性研究。评估了 TS-HDS/FGFR3 抗体与 SFN 标志物（包括表皮神经纤维密度（ENFD）、汗腺神经纤维密度（SGNFD）和瓦尔萨尔瓦动作、深呼吸、出汗和倾斜试验评估的自主功能障碍）之间的相关性。
在 322 名患者中；28%的患者抗 TS-HDS 升高，17%的患者抗 FGFR3 升高，96%的患者自主功能障碍，71%的患者 ENFD 异常，49%的患者 SGNFD 异常。无论皮肤活检是否正常，只要将 ENFD/SGNFD 结合起来用于抗 FGFR3 阳性，有自主神经功能障碍的患者都存在 TS-HDS/FGFR3 抗体。
有自主神经功能障碍证据的患者存在 TS-HDS/FGFR3 抗体。需要进一步研究来证明这些抗体在评估免疫介导的自主神经功能障碍中的临床价值。
