September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Fuchs’ endothelial corneal dystrophy: a systematic immunofluorescence study of Collagen type VIII suggests heterogeneous pathophysiology
Author Affiliations & Notes
  • Esben Nielsen
    Ophthalmology, Aarhus University Hospital, Aarhus C, Denmark
  • Jesper Hjortdal
    Ophthalmology, Aarhus University Hospital, Aarhus C, Denmark
  • Anders Ivarsen
    Ophthalmology, Aarhus University Hospital, Aarhus C, Denmark
  • Footnotes
    Commercial Relationships   Esben Nielsen, None; Jesper Hjortdal, None; Anders Ivarsen, None
  • Footnotes
    Support  Fight for Sight Denmark, Synoptik-Fonden, Jochum Jensen og hustru Mette Marie Jensens f. Poulsens mindelegat, Helga og Peter Kornings Fond, Købmand Marie Kirstine Jensens Fond, Novartis
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 5271. doi:
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    • Get Citation

      Esben Nielsen, Jesper Hjortdal, Anders Ivarsen; Fuchs’ endothelial corneal dystrophy: a systematic immunofluorescence study of Collagen type VIII suggests heterogeneous pathophysiology
      . Invest. Ophthalmol. Vis. Sci. 2016;57(12):5271.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : The purpose of this study was to investigate the pathophysiologic heterogeneity of Fuchs’ endothelial corneal dystrophy (FECD)

Methods : We conducted a systematic immunofluorescence study on 39 Descemet’s membrane (DM) samples from FECD patients and compared these to 10 DM samples from patients with pseudophakic bullous keratopathy (PBK) and 7 normal corneas. Samples were analyzed with immunofluorescence using antibodies to the α1-chain (COL8A1) and α2-chain (COL8A2) in collagen type VIII. Intensity of staining was assessed using a subjective grading scale from 0-3. The presence of specific staining patterns was noted.

Results : Two observers graded immunofluorescence images, blinded from each other’s results. Inter-observer agreement (weighted kappa) was 0.84, indicating a ‘very good’ agreement. FECD samples stained significantly more for the COL8A1 antibody when compared with samples in the PBK group (p=0.034), and samples in normal corneas (p=0.004). There was no difference in staining for COL8A2 antibody between the groups. Three different staining patterns were noticed: diffuse, refractile strands and lumpy. COL8A1 and COL8A2 displayed all three of these staining patterns in the FECD group. There were substantial variations in staining intensity and pattern in the FECD group, a phenomenon that was especially pronounced for the COL8A2 antibody. Negative controls did not stain.

Conclusions : We found increased staining for COL8A1, but not COL8A2 in FECD patients. This implies that Collagen VIII plays a role in FECD pathophysiology. Substantial variations in staining intensity and staining patterns in FECD patients were noticed, indicating pathophysiological heterogeneity. This supports results from genetic studies suggesting that the FECD phenotype may arise from several disease entities.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

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