From the Guidelines
Systemic sclerosis (SSc) can affect the eyes in several ways, including dry eyes, eyelid changes, and retinal abnormalities, with dry eyes being the most common ocular manifestation, occurring in up to 70% of patients due to fibrosis of lacrimal glands and reduced tear production 1.
Ocular Manifestations
The ocular manifestations of SSc can be diverse and affect various parts of the eye. Some of the key manifestations include:
- Dry eyes (keratoconjunctivitis sicca): This is the most common ocular manifestation, resulting from fibrosis of the lacrimal glands and subsequent reduction in tear production.
- Eyelid changes: These include tightening and retraction of the eyelids due to skin fibrosis, which may necessitate the use of lubricating ointments at night.
- Retinal abnormalities: These can include vascular changes such as cotton wool spots and retinal hemorrhages, particularly in patients with systemic hypertension or renal involvement.
Treatment and Management
Treatment for the ocular manifestations of SSc focuses on managing symptoms and preventing further complications. This can include:
- Artificial tears (preservative-free) used 4-6 times daily for dry eyes, with more severe cases potentially requiring punctal plugs or topical cyclosporine 0.05% twice daily.
- Regular ophthalmologic examinations every 6-12 months, with more frequent monitoring if symptoms develop, to ensure early detection and management of ocular manifestations.
Pathophysiology and Importance of Early Detection
The pathophysiology of SSc involves excessive collagen deposition, vascular damage, and autoimmune inflammation, which can affect various eye structures and lead to progressive damage if not managed properly 1. Early detection and management of these ocular manifestations are crucial to prevent vision loss and improve the quality of life for patients with SSc.
From the Research
Ocular Manifestations of Systemic Sclerosis (SSc)
The ocular manifestations of systemic sclerosis (SSc) can be diverse and affect various parts of the eye. Some of the common ocular manifestations include:
- Eyelid skin alterations, such as stiffness, tightness, or telangiectasia, which were found in 56.86% of patients in one study 2 and in 11 patients (stiffness or tightness) and 8 patients (telangiectasia) in another study 3
- Dry eye disease (DED), which was found in 64.71% of patients in one study 2 and in 14 cases in another study 3
- Retinal abnormalities, such as retinal vascular abnormalities, which were found in 50.98% of patients in one study 2 and in 20 eyes in another study 4
- Cataracts, such as posterior subcapsular cataract, which were found in 50.98% of patients in one study 2 and in 10 eyes in another study 4
- Conjunctival changes, such as injection or vascular sludging, which were found in 15.69% of patients in one study 2 and in 19 cases (injection) and 27 cases (vascular sludging) in another study 3
- Iris abnormalities, which were found in 13.73% of patients in one study 2 and in 6 cases (iris light reflux) in another study 3
- Glaucoma, which was found in 21.57% of patients in one study 2 and in 11 eyes (increased intraocular pressure) in another study 4
Anterior and Posterior Segment Involvement
The ocular manifestations of SSc can affect both the anterior and posterior segments of the eye. The anterior segment can be affected by:
- Eyelid skin remodeling
- Dry eye syndrome
- Conjunctival abnormalities The posterior segment can be affected by:
- Retinal microcirculatory system abnormalities
- Optic nerve abnormalities
- Vascular abnormalities within the fundus 5, 4
Prevalence of Ocular Manifestations
The prevalence of ocular manifestations in SSc patients can vary. One study found that 24 out of 750 patients (3.2%) had significant ocular involvement 6. Another study found that 27 patients with SSc had a higher incidence of ocular abnormalities, including dry eye syndrome, astigmatism, posterior subcapsular cataract, increased intraocular pressure, and vascular abnormalities within the fundus, compared to a control group of 27 healthy individuals 4.