Affordable Access

Access to the full text

Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study

Authors
  • Triggianese, Paola1
  • Cesareo, Massimo1
  • Guarino, Maria Domenica1
  • Conigliaro, Paola1
  • Chimenti, Maria Sole1
  • Cedola, Francesca1
  • Mazzeo, Caterina1
  • Nucci, Carlo1
  • Perricone, Roberto1
  • 1 University of Rome Tor Vergata, Rome, Italy , Rome (Italy)
Type
Published Article
Journal
Orphanet Journal of Rare Diseases
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Jan 17, 2020
Volume
15
Issue
1
Identifiers
DOI: 10.1186/s13023-019-1263-6
Source
Springer Nature
Keywords
License
Green

Abstract

Evidence supports that hereditary angioedema (HAE) may be considered as a paroxysmal permeability disorder with defective but self-limiting endothelial barrier dysfunction. A potential subclinical abnormal vascular permeability at retinal capillaries could induce damage resulting in retinopathy. We aimed at exploring for the first time the presence of microangiopathy at retinal level from a highly selective cohort of patients with HAE due to C1 esterase inhibitor protein (C1INH) deficiency (type I). We conducted a pilot, prospective, case-control study including 20 type I HAE patients and 20 age−/sex-matched healthy controls (HC). All participants underwent standard ophthalmological examination including visual fields. Superficial and deep capillary plexi in the retina were analyzed by using new optical coherence tomography angiography (OCT-A). A total of 40 eyes from 20 HAE patients and 20 eyes from HC were evaluated. Perimetric indices of visual field were slightly worse in HAE than in controls. OCT-angiograms documented in HAE patients a lower retinal capillary density in both superficial and deep scans and a higher retinal thickness compared to healthy eyes. Our findings firstly documented subclinical abnormalities in retinal microvascular network in type I HAE patients that might be associated with early subtle functional changes. This preliminary evidence supports the hypothesis of a recurrent endothelial barrier failure at retinal level in HAE patients potentially resulting in chronic damage.

Report this publication

Statistics

Seen <100 times