Scleroderma, formally known as Systemic Sclerosis (SSc), is a chronic autoimmune disease that affects the connective tissue throughout the body. The condition is characterized by three processes: an abnormal immune response, progressive fibrosis, and widespread damage to small blood vessels (vasculopathy). While it is most noticeable for causing the hardening and tightening of the skin, the disease is systemic, meaning it can affect internal organs like the lungs, heart, kidneys, and the eyes. Ocular involvement is a recognized complication of scleroderma, resulting from both the direct effects of tissue fibrosis and the systemic microvascular changes that characterize the disease.
The Most Common Manifestation: Dry Eyes and Eyelid Changes
The most frequent ocular issues involve the surface of the eye, often presenting as a severe form of chronic dry eye disease (keratoconjunctivitis sicca). This dryness results from deficiencies in the tear film. Fibrosis can affect the lacrimal glands, decreasing the aqueous component of tears, and reducing conjunctival goblet cells limits the amount of protective mucin.
Dry eye syndrome is highly prevalent in people with scleroderma. Severe dryness is often linked to secondary Sjögren’s syndrome, an overlapping autoimmune disorder. Tear film instability is also exacerbated by meibomian gland dysfunction, which normally secretes the lipid layer that prevents tear evaporation.
Fibrotic changes in the skin surrounding the eyes also contribute to surface problems. The tightening and rigidity of the eyelid skin can impair the natural blinking motion. This stiffness can lead to lagophthalmos (inability to fully close the eyelids) or blepharophimosis (narrowing of the eyelid opening). These structural changes prevent tears from being evenly distributed, accelerating evaporation and worsening dryness.
The conjunctiva, the membrane covering the white of the eye, may show scleroderma-related vascular changes. Small, dilated blood vessels called telangiectasias are sometimes visible on the eyelids or conjunctiva. These findings mirror the microvascular abnormalities seen elsewhere in the body.
Deeper Systemic Impact on Internal Ocular Structures
Systemic vasculopathy and connective tissue damage characteristic of scleroderma can affect the internal structures of the eye, though these complications are generally less common than dry eyes. The posterior segment, which includes the retina and choroid, is particularly susceptible to microvascular disease, leading to retinal or choroidal vasculopathy.
Advanced imaging reveals significant impairment of the choriocapillaris and retinal vascular plexuses, even early in the disease. These changes manifest as thinning of choroidal capillaries, areas of ischemia (lack of blood flow), and microaneurysms within the retina. Although often asymptomatic initially, this damage can lead to vision compromise.
A severe threat to internal structures is linked to systemic hypertension, especially during scleroderma renal crisis. Uncontrolled high blood pressure can trigger hypertensive retinopathy, damaging retinal vessels and sometimes affecting the optic nerve. Optic neuropathy (damage to the nerve connecting the eye to the brain) can occur, potentially leading to optic atrophy and vision loss.
Scleroderma may also cause inflammatory conditions like uveitis (inflammation of the uvea). Damage to connective tissue can increase the risk of glaucoma, including normal tension glaucoma. The high collagen content makes eye tissues vulnerable to the disease’s fibrotic processes.
Screening and Treatment Approaches for Eye Complications
Regular comprehensive ophthalmological examinations are necessary for individuals with scleroderma. These specialized eye exams can detect subtle changes before they cause permanent damage.
To assess for dry eye disease, ophthalmologists commonly use tests like the Schirmer’s test, which measures tear production, and the tear break-up time (TBUT), which evaluates the stability of the tear film. Fundoscopy allows visualization of the retinal and choroidal vasculature to check for signs of vasculopathy or hypertensive changes. Newer techniques, such as Optical Coherence Tomography Angiography (OCTA), provide detailed, non-invasive imaging of retinal microcirculation to track vascular damage progression.
Management strategies for common surface complications focus on maintaining lubrication and protecting the ocular surface. The first line of treatment involves consistent use of preservative-free artificial tears and lubricating gels. For severe dryness, devices like punctal plugs can be inserted to block tear drainage ducts, keeping tears on the eye surface longer. Eyelid hygiene, including warm compresses, is recommended to manage meibomian gland dysfunction.
Addressing severe internal complications requires managing the underlying systemic disease. Treatment for retinal vasculopathy or optic nerve involvement often involves coordinating care with a rheumatologist to ensure the systemic condition, especially any active inflammation or hypertension, is adequately controlled. Immunosuppressive medications and blood pressure-lowering drugs are often used to slow the progression of the disease and protect the internal ocular structures from further damage.