Scleroderma, or systemic sclerosis (SSc), is a chronic, autoimmune connective tissue disease characterized by three main processes: immune system dysfunction, microvascular damage, and excessive production of collagen. This overproduction of collagen leads to fibrosis, or tissue hardening and scarring, which affects the skin and internal organs throughout the body. While the disease is primarily known for its effects on the skin, lungs, and kidneys, it frequently involves the eyes and surrounding structures. Ocular involvement is common, often manifesting as uncomfortable surface symptoms, though severe vision loss directly caused by the disease is relatively rare.
Manifestations on the Ocular Surface and Eyelids
The most frequent and noticeable ocular complication of Scleroderma is severe Dry Eye Syndrome, technically known as keratoconjunctivitis sicca. This condition arises because the disease process targets the body’s moisture-producing glands responsible for tear production. Fibrosis and inflammation impair the function of these glands, resulting in an insufficient quantity of the aqueous layer of the tear film. This lack of lubrication causes symptoms such as a scratchy, foreign body sensation, redness, and sensitivity to light.
The characteristic skin hardening associated with Scleroderma also directly affects the delicate tissues around the eye. Excessive collagen deposition in the dermis of the face and eyelids causes the skin to become tight and stiff. This eyelid fibrosis is a highly common finding, reported in a majority of patients.
The tightening of the eyelid skin can restrict the range of motion and alter the natural contour of the eyelid itself. This often impairs the ability to fully close the eye, a condition called lagophthalmos, which leaves the ocular surface exposed. An incomplete blink prevents the tear film from being evenly spread across the cornea and conjunctiva, exacerbating the already severe dry eye symptoms.
The combination of reduced tear production and mechanical dysfunction from skin tightening makes the surface of the eye vulnerable to chronic irritation and potential damage. Furthermore, the small blood vessels in the conjunctiva, the clear membrane covering the white of the eye, can also display changes such as telangiectasias or vascular congestion due to the disease’s underlying vasculopathy.
Internal and Vascular Changes in the Eye
Beyond the surface structures, Scleroderma’s systemic nature means it can affect the internal, or posterior, structures of the eye, particularly those rich in blood vessels. The hallmark microvascular damage, or vasculopathy, that affects organs like the kidneys and lungs can also impact the retina and choroid. The choroid is a layer of blood vessels situated between the retina and the outer layer of the eye, supplying oxygen and nutrients to the outer retina.
Damage to the small arteries and capillaries here can lead to areas of retinal ischemia, where blood flow is restricted. This lack of adequate blood supply may manifest as retinal abnormalities observed in a significant number of Scleroderma patients. Specific signs include cotton wool spots, which are small, fluffy white patches on the retina representing nerve fiber layer damage from localized ischemia.
These internal changes are often linked to the severity of the overall systemic disease and related complications, such as high blood pressure. Severe, uncontrolled hypertension, particularly in the context of Scleroderma Renal Crisis, can dramatically worsen microvascular damage in the eye, sometimes leading to more widespread retinal damage. Although less common, the optic nerve may also be affected as a secondary complication of severe systemic disease or extremely high blood pressure.
The use of advanced imaging techniques, such as Optical Coherence Tomography Angiography (OCTA), has demonstrated significant impairment in the choriocapillaris, the dense network of blood vessels in the choroid. These findings highlight that microvascular impairment in the eye often mirrors the vascular problems occurring in other vital organs.
Ophthalmic Screening and Management Strategies
Given the wide range of potential ocular involvement, regular ophthalmic screening is important for individuals diagnosed with Scleroderma. A comprehensive eye examination allows for the early detection and management of both common surface issues and less frequent, but more serious, internal complications. Screening typically involves a slit lamp examination to assess the anterior segment, checking for signs of dry eye and eyelid changes.
Specific tests are used to quantify the severity of dry eyes, such as the Schirmer’s test, which measures tear production. For internal assessment, a fundoscopy is performed to view the retina and optic nerve, allowing the eye doctor to look for signs of microvascular damage, like cotton wool spots or choroidal thinning.
Ocular Surface Management
Management of dry eye typically begins with frequent use of preservative-free artificial tears to supplement the deficient natural tear film. If over-the-counter drops are insufficient, specialized treatments are necessary. Managing the effects of eyelid tightness also requires a personalized approach to protect the cornea.
Treatment strategies for the ocular surface include:
- Prescription anti-inflammatory drops.
- Specialized moisture-retaining contact lenses.
- Punctal plugs, which can be temporarily or permanently inserted into the tear drainage ducts to keep tears on the eye’s surface longer.
- Lubricating ointments used at night to prevent exposure keratopathy due to incomplete eyelid closure.
Systemic Disease Control
Systemic management of the underlying disease is equally important, requiring close collaboration between the ophthalmologist and the patient’s rheumatologist. Controlling the autoimmune process and vasculopathy through immunosuppressive or vasoactive therapies is the most effective way to protect the retina and choroid from further damage.