Corneal confocal microscopy detects corneal nerve damage and increased dendritic cells in Fabry disease

Gulfidan Bitirgen, Kultigin Turkmen, Rayaz Malik, Ahmet Ozkagnici, Nazmi Zengin

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Fabry disease is characterised by neuropathic pain and accelerated vascular disease. This study evaluates the utility of corneal confocal microscopy (CCM) to non-invasively quantify corneal nerve and endothelial cell morphology and dendritic cell (DC) density in relation to disease severity in subjects with Fabry disease. Seventeen consecutive participants with Fabry disease and 17 healthy control subjects were included in this cross-sectional study. Fabry disease severity was measured using the Mainz Severity Score Index (MSSI). Central corneal sensitivity was assessed with a contact corneal esthesiometer. There was a significant reduction in the corneal sensitivity (5.75 [5.25–6.00] vs. 6.00 [6.00-6.00] cm, P = 0.014), nerve fiber density (NFD) (26.4 ± 10.1 vs. 33.7 ± 7.9 fibers/mm2, P = 0.025) and nerve fiber length (NFL) (15.9 ± 3.4 vs. 19.5 ± 4.4 mm/mm2, P = 0.012) and an increase in DC density (38.3 [17.5–97.3] vs. 13.5 [0–29.4] cells/mm2, P = 0.004) in subjects with Fabry disease compared to the healthy control subjects. The total MSSI score correlated with NFD (ρ = −0.686; P = 0.006), NFL (ρ = −0.692; P = 0.006), endothelial cell density (ρ = −0.511; P = 0.036), endothelial cell area (ρ = 0.514; P = 0.036) and α-galactosidase A enzyme activity (ρ = −0.723; P = 0.008). This study demonstrates reduced corneal sensitivity, corneal nerve fiber damage and increased DCs in subjects with Fabry disease.

Original languageEnglish
Article number12244
JournalScientific Reports
Volume8
Issue number1
DOIs
Publication statusPublished - 1 Dec 2018

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Fabry Disease
Confocal Microscopy
Dendritic Cells
Nerve Fibers
Endothelial Cells
Cell Count
Healthy Volunteers
Galactosidases
Neuralgia
Vascular Diseases
Corneal Injuries
Cross-Sectional Studies
Neurons
Enzymes

ASJC Scopus subject areas

  • General

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Corneal confocal microscopy detects corneal nerve damage and increased dendritic cells in Fabry disease. / Bitirgen, Gulfidan; Turkmen, Kultigin; Malik, Rayaz; Ozkagnici, Ahmet; Zengin, Nazmi.

In: Scientific Reports, Vol. 8, No. 1, 12244, 01.12.2018.

Research output: Contribution to journalArticle

Bitirgen, Gulfidan ; Turkmen, Kultigin ; Malik, Rayaz ; Ozkagnici, Ahmet ; Zengin, Nazmi. / Corneal confocal microscopy detects corneal nerve damage and increased dendritic cells in Fabry disease. In: Scientific Reports. 2018 ; Vol. 8, No. 1.
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