From the Guidelines
Pseudoexfoliation syndrome (PXF) is a condition characterized by the deposition of white, dandruff-like material on the anterior lens capsule, pupillary margin, and other anterior segment structures, and is associated with an increased risk of pseudoexfoliation glaucoma, cardiovascular and cerebrovascular disease, and other systemic conditions. The ocular manifestations of PXF include:
- White, dandruff-like material deposited on the anterior lens capsule, pupillary margin, and other anterior segment structures
- Pigment dispersion on the corneal endothelium
- Iris transillumination defects
- Poor pupillary dilation
- Zonular weakness leading to lens subluxation
- Increased risk of complications during cataract surgery The most significant ocular association is pseudoexfoliation glaucoma, which develops in approximately 40% of patients with PXF and is characterized by higher intraocular pressures, more severe optic nerve damage, and greater resistance to medical therapy than primary open-angle glaucoma 1. Systemically, PXF is associated with:
- Cardiovascular and cerebrovascular disease, including hypertension, angina, myocardial infarction, stroke, and abdominal aortic aneurysms
- Hearing loss
- Alzheimer's disease
- Transient ischemic attacks The condition results from abnormal production and accumulation of an elastin-related microfibrillar material, with both genetic factors (particularly LOXL1 gene mutations) and environmental influences contributing to its development 1. PXF is more common in older individuals and varies in prevalence across different ethnic populations. It is essential to monitor patients with PXF for the development of pseudoexfoliation glaucoma and other systemic conditions, and to manage these conditions promptly to prevent long-term morbidity and mortality 1.
From the Research
Ocular Manifestations of Pseudoexfoliation Syndrome
- Pseudoexfoliation syndrome (PES) is a systemic disorder that affects the eyes, leading to increased intraocular pressure, changes in the anatomical aspects of the optic nerve, and visual field alterations 2.
- The syndrome is characterized by the accumulation of abnormal fibrillar extracellular material in the anterior chamber of the eye, which can be detected by ocular slit-lamp examination 3.
- PES can lead to pseudoexfoliation glaucoma (PEG), a condition with a higher risk of vision loss, higher maximum and mean intraocular pressure, and a wider range of IOP fluctuation compared to primary open-angle glaucoma 4.
- Other ocular manifestations of PES include zonular weakness, lens subluxation or dislocation, and an increased incidence of cataract development 4.
Systemic Associations of Pseudoexfoliation Syndrome
- PES has been associated with various systemic conditions, including cardiovascular, cerebrovascular, and abdominal aorta aneurysm diseases 5.
- There is also growing evidence of associations with peripheral, cardiovascular, and cerebrovascular system diseases, Alzheimer's disease, hearing loss, and increased plasma homocysteine levels 4.
- PES has been linked to increased vascular risk, risk of dementia, and inflammatory state, suggesting that it may be a marker for systemic disease 3.
- The extracellular material found in PES has also been detected in other parts of the body, including the skin, heart, lungs, liver, and kidneys, highlighting the systemic nature of the condition 3.
Pathogenesis and Treatment of Pseudoexfoliation Syndrome
- The pathogenesis of PES is thought to involve the dysregulation of extracellular matrix (ECM) homeostasis, leading to the accumulation of abnormal fibrillar material 6.
- Various stimuli, including oxidation stress, UV radiation, and osmotic pressure, can affect the progression of PES 6.
- Treatment options for PES and PEG include topical medical treatment, surgical techniques such as Argon Laser or Selective Laser Trabeculoplasty, and other surgical procedures to manage glaucoma and cataract development 2, 4.