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Lucia Pace, Federico Corvi, Andrea Giani, Lucia Lonati, emma zanzottera, Vacchini Romualdo, Marco Cicardi, Giovanni Staurenghi; Prevalence of reticular pseudodrusen and drusen in hereditary angioedema. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1498. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
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.
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 center, 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.
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.
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.
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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