June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Quantitative fundus autofluorescence associated with a diseased Bruch membrane in pseudoxanthoma elasticum
Author Affiliations & Notes
  • Martin Gliem
    University Eye Hospital Bonn, Bonn, Germany
    Center for Rare Diseases Bonn (ZSEB), Bonn, Germany
  • Philipp Mueller
    University Eye Hospital Bonn, Bonn, Germany
    Center for Rare Diseases Bonn (ZSEB), Bonn, Germany
  • Johannes Birtel
    University Eye Hospital Bonn, Bonn, Germany
    Center for Rare Diseases Bonn (ZSEB), Bonn, Germany
  • Robert P. Finger
    University Eye Hospital Bonn, Bonn, Germany
    Center for Rare Diseases Bonn (ZSEB), Bonn, Germany
  • Frank G Holz
    University Eye Hospital Bonn, Bonn, Germany
    Center for Rare Diseases Bonn (ZSEB), Bonn, Germany
  • Peter Charbel Issa
    Oxford Eye Hospital, Oxford, United Kingdom
  • Footnotes
    Commercial Relationships   Martin Gliem, Bayer (R), Carl Zeiss Meditec (F), Heidelberg Engineering (F), Optos (F); Philipp Mueller, Carl Zeiss Meditec (F), Heidelberg Engineering (F), Optos (F); Johannes Birtel, Carl Zeiss Meditec (F), Heidelberg Engineering (F), Optos (F); Robert Finger, Bayer (C), Novartis (C), Novartis (F), Opthea (C), QLT (C), Santen (C); Frank Holz, Acucela (F), Acucela (C), Alcon (F), Alcon (C), Allergan (F), Allergan (C), Bayer Health Care (C), Boehringer Ingelheim (C), Carl Zeiss Meditec (F), Genentec (C), Heidelberg Engineering (C), Heidelberg Engineering (R), Heidelberg Enginnering (F), Merz (C), Novartis (F), Novartis (C), Optos (F), Roche (C); Peter Charbel Issa, Heidelberg Engineering (F)
  • Footnotes
    Support  BONFOR research grant University of Bonn O-137.0018
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 3689. doi:
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      Martin Gliem, Philipp Mueller, Johannes Birtel, Robert P. Finger, Frank G Holz, Peter Charbel Issa; Quantitative fundus autofluorescence associated with a diseased Bruch membrane in pseudoxanthoma elasticum. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3689.

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

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Abstract

Purpose : To quantify lipofuscin-associated fundus autofluorescence in patients with a diseased Bruch membrane due to pseudoxanthoma elasticum (PXE).

Methods : For this prospective, monocenter, cross-sectional case-control study 49 patients with PXE (mean age 46 years, range 18-62) and 108 healthy controls (mean age 40 years, range 18-64) were investigated. All subjects underwent quantitative fundus autofluorescence (qAF) imaging with a modified scanning laser ophthalmoscope equipped with an internal fluorescent reference (modified Spectralis HRA-OCT, Heidelberg Engineering) to account for variations of laser power and detector sensititvity. The mean qAF values of a circular ring centered to the fovea (qAF8) were measured and compared between groups.

Results : Overall, patients with PXE showed reduced qAF8 values compared to controls (difference from controls -23%, 95% confidence interval (CI) -29 to -16%, p<0.001). The reduction was most pronounced in the subgroup between 40-60 years (-36%, 95% CI -42 to -30%, p<0.001) and was negatively correlated with the extent of Bruch membrane calcification (r=-0.51, 95% CI -0.69 to -0.26), measured as the eccentricity of the most central border of peau d’orange. Of the 13 cases below 40 years of age, 12 (92%) showed qAF8 values within normal limits. Topographically, patients with PXE showed relatively higher values in temporal and relatively lower values in nasal segments. The phenotype of patients with reduced qAF values was characterized by pattern-dystrophy-like changes (70%; 21 of 29) and reticular pseudodrusen (60%; 18 of 29).

Conclusions : Reduced qAF8 values are a characteristic finding in patients with PXE. The association with pathology of Bruch membrane expands the pathophysiologic understanding of PXE and of multifactorial diseases with pathology of Bruch’s membrane, such as age-related macular degeneration.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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