Prevalence of reticular pseudodrusen and drusen in hereditary angioedema

Session Details

Session Title: Free Paper Session 4: AMD II

Session Date/Time: Thursday 07/09/2017 | 11:00-12:30

Paper Time: 11:54

Venue: Room 111

First Author: : L.Pace ITALY

Co Author(s): :    F. Corvi   A. Giani   L. Lontani   E. Zanzottera   M. Cicardi   G. Staurenghi              

Abstract Details

Purpose:

Reticular pseudodrusen (RPD) and drusen are considered early signs of age related macular degeneration (AMD). This disease is correlated with some genetic, environmental and immunologic factors. In particular, complement system plays an important role in retinal biology and its deficiency can worsen retinal homeostasis. Our purpose is to describe prevalence, features, and topographic distribution of RPD and retinal changes in patients with hereditary angioedema.

Setting:

Retrospective observational case series

Methods:

Consecutive patients with hereditary angioedema underwent spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), and infrared reflectance (IR). The enrollment period was 12 months, from January to December 2012. It was an observational, single centre, cohort study. Inclusion criteria were informed consent, adult patients of both sexes of 39 or more years and established diagnosis of hereditary angioedema. Exclusion criteria were media opacity and inability to undergo imaging exams.

Results:

A total of 148 eyes from 74 patients with hereditary angioedema were enrolled. There were 35 male and 39 female with a mean age of 55 ± 11,8 years. 9 eyes (6%) showed retinal changes, in particular RPD were detected in 5 eyes (3,3%), both drusen and RPD in 2 eyes (1,3%), only drusen in 2 eyes (1,3%). Mean age of these patients was of 68,6 ±11,76 years. RPD were most frequently located in the nasal-external and superior-internal areas of the Early Treatment Diabetic Retinopathy Study quadrants; the inferior-internal zone was less interested. Among the patients with RPD, female was the most common gender (75%). RPD were identified as subretinal deposits on spectral-domain optical coherence tomographic images, they appeared hypoautofluorescent on fundus autofluorescence and hyporeflective on infrared reflectance.

Conclusions:

Early signs of AMD, as RPD and drusen, did not show a high prevalence among patients with hereditary angioedema, despite this disease is characterized by a deficiency in the C1 inhibitor. Patients with RPD and drusen were older than patients without these features. Relying on the above data, it seems there is no connection with AMD. The main risk factor linked to the pathology seems to be age: older patients are more likely to incur the pathology. This trend is also observed in the common population.

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