Small-Vessel Vasculitis or Perifolliculitis in Small-Fiber Neuropathy With TS-HDS, FGFR-3, or Plexin D1 Antibodies.

Q3 Medicine Journal of Clinical Neuromuscular Disease Pub Date : 2024-12-01 DOI:10.1097/CND.0000000000000492

Lawrence A Zeidman, Todd Levine, John Cangelosi

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Abstract

Introduction: Small-fiber neuropathy (SFN) is highly prevalent but often idiopathic. TS-HDS, FGFR-3, and Plexin D1 autoantibodies (seropositive) may be present in more than 40% of idiopathic cases. Another autoimmune biomarker is a non-length-dependent (NLD) skin biopsy pattern. Our goal was to demonstrate that small-vessel vasculitis and perifolliculitis (inflammation) on skin biopsies are additional biomarkers.

Methods: All pure SFN skin biopsy reports were reviewed for inflammation, and their charts were examined for other relevant history.

Results: Seven of 80 patients with pure SFN had inflammation (8.8%); 5 patients were female (71%) and 2 were male (29%); average age was 45 (16-67). All 7 patients with inflammation were seropositive (100%, P = 0.0495), and 6 patients (86%) had either NLD inflammation or NLD pathology (P = 0.0003).

Discussion: Inflammation is present only in a small portion of punch biopsies, but may be another autoimmune SFN biomarker. It is strongly associated with seropositivity and NLD-pathology. Further studies are likely indicated to assess inflammation pathophysiology and immunotherapy responsiveness.

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带有 TS-HDS、FGFR-3 或 Plexin D1 抗体的小纤维神经病中的小血管炎或毛周炎

简介小纤维神经病（SFN）发病率很高，但通常是特发性的。40%以上的特发性病例可能存在 TS-HDS、FGFR-3 和 Plexin D1 自身抗体（血清阳性）。另一种自身免疫生物标志物是非长度依赖性（NLD）皮肤活检模式。我们的目标是证明皮肤活检中的小血管炎和毛囊周围炎（炎症）是额外的生物标志物：方法：对所有纯SFN皮肤活检报告进行炎症审查，并检查病历以了解其他相关病史：结果：80 名单纯性 SFN 患者中有 7 人（8.8%）患有炎症；其中 5 人为女性（71%），2 人为男性（29%）；平均年龄为 45 岁（16-67 岁）。7名炎症患者的血清反应均为阳性（100%，P = 0.0495），6名患者（86%）有NLD炎症或NLD病变（P = 0.0003）：讨论：炎症只出现在一小部分冲孔活检中，但可能是另一种自身免疫性 SFN 生物标志物。它与血清阳性和 NLD 病理密切相关。进一步的研究可能有助于评估炎症的病理生理学和免疫疗法的反应性。

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来源期刊

Journal of Clinical Neuromuscular Disease Medicine-Medicine (all)

CiteScore

1.60

自引率

0.00%

发文量

期刊介绍： Journal of Clinical Neuromuscular Disease provides original articles of interest to physicians who treat patients with neuromuscular diseases, including disorders of the motor neuron, peripheral nerves, neuromuscular junction, muscle, and autonomic nervous system. Each issue highlights the most advanced and successful approaches to diagnosis, functional assessment, surgical intervention, pharmacologic treatment, rehabilitation, and more.