Abstract
Pseudoexfoliation syndrome (PES) is one of the most common causes of open-angle glaucoma, with a higher risk of vision loss, a higher maximum and mean intraocular pressure (IOP) at diagnosis, and a wider range of IOP fluctuation compared to primary open-angle glaucoma. Patients with this syndrome have a ten-fold higher risk of developing glaucoma than the normal population. A definite diagnosis can be made by the observation of pseudoexfoliation material (PEM) on the anterior lens surface, ciliary processes, zonules, and iris. PEM deposits on the zonules may explain the clinically observed zonular weakness and lens subluxation or dislocation. An increased incidence of cataract development is also associated with PES. There is growing evidence for systemic associations of PES with peripheral, cardiovascular, and cerebrovascular system diseases, Alzheimer’s disease, hearing loss, and increased plasma homocysteine levels. Indications for surgery are markedly more common in patients with pseudoexfoliation glaucoma than primary open-angle glaucoma. The goal of this article is to review the latest perspectives on the clinical features, therapy, and systemic associations of this clinically and biologically challenging disease.
Keywords: Pseudoexfoliation, pseudoexfoliation syndrome, pseudoexfoliation glaucoma
Introduction
Pseudoexfoliation syndrome (PES) is the most common identified cause of open-angle glaucoma. Pseudoexfoliation is an independent risk factor for open-angle glaucoma in every country around the world. It is important to distinguish pseudoexfoliation glaucoma (PEG) from primary open-angle glaucoma (POAG). PEG is a severe type of glaucoma with a higher risk of vision loss, higher maximum and mean intraocular pressure (IOP) at diagnosis, and wider range of IOP fluctuation compared to POAG.
PEG may cause increased outflow resistance as a result of progressive accumulation of pseudoexfoliation material (PEM) in the trabecular meshwork, which contributes to alteration of retrobulbar blood flow and optic nerve microvascular blood flow, as well as elastosis of the lamina cribrosa.
A definite diagnosis can be made by observing PEM on the anterior lens surface. These deposits can be found initially on the ciliary processes, zonules, and iris. Deposits of pseudoexfoliation on the zonules may explain the clinically observed zonular weakness and lens subluxation or dislocation. An increased incidence of cataract development is also associated with PES.
PES may be an important marker for cardiovascular and cerebrovascular diseases. There is growing evidence for systemic associations of PES with peripheral, cardiovascular, and cerebrovascular system diseases, Alzheimer’s disease, hearing loss, and increased plasma homocysteine levels.,
Clinical experiences show that controlling IOP is more difficult in PEG than in POAG. PEG patients with high IOP and advanced damage may benefit from a combination of anti-glaucoma medications as initial therapy. The indications for surgery are markedly more common in patients with PEG than in POAG.
The purpose of this article is to review current perspectives on the clinical features, therapy, and systemic associations of this clinically and biologically challenging disease.
Conclusions
PES and glaucoma are public health problems in older age worldwide. In recent years, the expansion in knowledge of the epidemiology, pathogenesis, and genetics of PES and glaucoma has provided important insights for understanding this disease and future treatment.
Pseudoexfoliation is an age-dependent and stress-induced extracellular fibrotic matrix disorder in which the oxidative-antioxidative balance is disturbed. LOXL1 is involved in the pathogenesis. Patients with PES have a higher risk of cardiovascular and cerebrovascular diseases.
The presence of pseudoexfoliation is an important risk factor for glaucoma and cataract. It is necessary to distinguish PEG from POAG. Medical management is more difficult and surgery is required more frequently. Early diagnosis, appropriate treatment and more frequent examinations appear to prevent the progression of visual field and vision loss.
In the future, new research can increase our understanding of the epidemiology, pathogenesis, genetics, and classifications of this disease. New therapeutic approaches will be considered for its management.
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