Abstract
Purpose :
To assess the systemic associations of angioid streaks (AS) using a large U.S. healthcare database.
Methods :
This is a retrospective cross-sectional study of all patients diagnosed with AS, identified by ICD9 and ICD10 coding, in a large, national U.S. insurer from 2000 - 2019. All patients were 18 years or older and had at least one active year of enrollment within the plan. Any patient with a diagnosis of choroidal rupture or pathologic myopia were excluded. The literature was reviewed for all published associations with AS, as well as those in the standard mnemonic for AS (PEPSI). The rates of each disease state in those with AS were then calculated.
Results :
After applying exclusion criteria, 2,072 patients were eligible for the study. The mean and standard deviation of age for this group is 63.4 (18.5) years, respectively, with 817 (39.4%) males and 1,255 (60.6%) females. The number of patients and rates of association between AS and the well characterized conditions of the PEPSI mnemonic are as follows: Pseudoxanthoma elasticum (PXE) - 258 patients (12.5%), Ehlers-Danlos syndrome - 21 patients (1.0%), Paget’s disease - 7 patients (0.3%), hemoglobinopathies (i.e. sickle cell disease) - 33 patients (1.6%), and idiopathic, the largest group -1,756 patients (84.7%). To enumerate the less commonly described but previously reported diseases associated with AS, 514 patients (24.8%) were diagnosed with diabetes, 319 patients (15.4%) familial adenomatous polyposis, 222 patients (10.7%) had lead poisoning, 524 (25.3%) had senile elastosis, and 697 (33.6%) had non-exudative AMD.
Conclusions :
AS, pathologically characterized as breaks in Bruch’s membrane, has a known significant association with PXE. Concurrently, the association of AS with other less reported diseases lends support to a new mnemonic and new mechanistic understanding of AS formation.
This is a 2021 ARVO Annual Meeting abstract.