Corneal neuro-immune crosstalk in Fabry disease: An in vivo confocal microscopic study

IF 2.5 4区医学 Q3 IMMUNOLOGY

Journal of neuroimmunology Pub Date : 2025-09-16 DOI:10.1016/j.jneuroim.2025.578759

Xuecong Zhou , Yingsi Li , Yawen Zhao , Xiaoming Yan , Wei Zhang , Yuan Wu

{"title":"Corneal neuro-immune crosstalk in Fabry disease: An in vivo confocal microscopic study","authors":"Xuecong Zhou , Yingsi Li , Yawen Zhao , Xiaoming Yan , Wei Zhang , Yuan Wu","doi":"10.1016/j.jneuroim.2025.578759","DOIUrl":null,"url":null,"abstract":"<div><h3>Purpose</h3><div>This study aims to assess the corneal nerve damage and corneal immune alteration by <em>in vivo</em> confocal microscopy (IVCM) in Fabry disease (FD)</div></div><div><h3>Methods</h3><div>Sixty-two eyes from 31 patients with FD were analysed and compared with fifty eyes from 25 healthy controls in this prospective, cross-sectional, controlled, single-center study. After evaluating corneal sensation by the Cochet-bonnet esthesiometer, the IVCM was performed to evaluate the sub-basal nerve plexus (CSNP) including density, number, tortuosity and reflectivity of corneal nerve, and the inflammatory cells including mature/immature Langerhans cells (LCs) and leukocytes. The differences between FD and healthy controls, different genders (heterozygous and hemizygous), phenotypes (classical, nonclassical) and Mainz severity score index (MSSI) scores were compared</div></div><div><h3>Results</h3><div>A significant reduction of corneal nerve density, number and reflectivity, as well as an increase of tortuosity occurred in the FD group compared with healthy controls (<em>P</em> < 0.001). The density of LCs was significantly increased in the FD group compared with the healthy controls in the central cornea (<em>P</em> = 0.016) and peripheral cornea (<em>P</em> < 0.001). The more severe corneal neuropathy and higher density of LCs were found in hemizygous males than heterozygous females (<em>P</em> < 0.05). As for the difference between distinct phenotypes and MSSI scores, we did not find significant difference in corneal nerve parameters and LCs density</div></div><div><h3>Conclusions</h3><div>IVCM provides parameters that reliably indicate corneal nerve damage and inflammatory activation in patients with FD</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"409 ","pages":"Article 578759"},"PeriodicalIF":2.5000,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of neuroimmunology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0165572825002401","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"IMMUNOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Purpose

This study aims to assess the corneal nerve damage and corneal immune alteration by in vivo confocal microscopy (IVCM) in Fabry disease (FD)

Methods

Sixty-two eyes from 31 patients with FD were analysed and compared with fifty eyes from 25 healthy controls in this prospective, cross-sectional, controlled, single-center study. After evaluating corneal sensation by the Cochet-bonnet esthesiometer, the IVCM was performed to evaluate the sub-basal nerve plexus (CSNP) including density, number, tortuosity and reflectivity of corneal nerve, and the inflammatory cells including mature/immature Langerhans cells (LCs) and leukocytes. The differences between FD and healthy controls, different genders (heterozygous and hemizygous), phenotypes (classical, nonclassical) and Mainz severity score index (MSSI) scores were compared

Results

A significant reduction of corneal nerve density, number and reflectivity, as well as an increase of tortuosity occurred in the FD group compared with healthy controls (P < 0.001). The density of LCs was significantly increased in the FD group compared with the healthy controls in the central cornea (P = 0.016) and peripheral cornea (P < 0.001). The more severe corneal neuropathy and higher density of LCs were found in hemizygous males than heterozygous females (P < 0.05). As for the difference between distinct phenotypes and MSSI scores, we did not find significant difference in corneal nerve parameters and LCs density

Conclusions

IVCM provides parameters that reliably indicate corneal nerve damage and inflammatory activation in patients with FD

Abstract Image

查看原文本刊更多论文

法布里病的角膜神经免疫串扰：体内共聚焦显微镜研究

目的利用体内共聚焦显微镜（IVCM）观察法布里病（FD）患者角膜神经损伤及角膜免疫功能改变。方法采用前瞻性、横断面、对照、单中心研究，对31例FD患者62只眼进行分析，并与25例健康对照者50只眼进行比较。在用Cochet-bonnet感觉仪评估角膜感觉后，采用IVCM评估基底下神经丛（CSNP），包括角膜神经的密度、数量、弯曲度和反射率，以及炎症细胞包括成熟/未成熟朗格汉斯细胞（LCs）和白细胞。比较FD组与健康对照组、不同性别（杂合子和半合子）、表型（经典型、非经典型）和Mainz严重性评分指数（MSSI）评分的差异。结果FD组与健康对照组相比，角膜神经密度、数量和反射率显著降低，扭曲度显著增加（P < 0.001）。与健康对照组相比，FD组角膜中央（P = 0.016）和周围（P < 0.001）的LCs密度显著增加。半合子男性比杂合子女性角膜神经病变更严重，LCs密度更高（P < 0.05）。对于不同表型和MSSI评分之间的差异，我们没有发现角膜神经参数和LCs密度有显著差异。结论sivcm提供了可靠的FD患者角膜神经损伤和炎症激活的参数

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Journal of neuroimmunology 医学-免疫学

CiteScore

6.10

自引率

3.00%

发文量

154

审稿时长

37 days

期刊介绍： The Journal of Neuroimmunology affords a forum for the publication of works applying immunologic methodology to the furtherance of the neurological sciences. Studies on all branches of the neurosciences, particularly fundamental and applied neurobiology, neurology, neuropathology, neurochemistry, neurovirology, neuroendocrinology, neuromuscular research, neuropharmacology and psychology, which involve either immunologic methodology (e.g. immunocytochemistry) or fundamental immunology (e.g. antibody and lymphocyte assays), are considered for publication.