Systemic Sclerosis, commonly known as Scleroderma, is a chronic autoimmune connective tissue disease characterized by generalized vasculopathy, a dysfunctional immune response, and progressive fibrosis. This combination leads to the thickening and hardening of the skin and internal organs, including the lungs, heart, and kidneys. The structures of the eye are also frequently involved, reflecting the high collagen content in ocular tissues and their extensive microcirculatory network. Ocular manifestations can affect both the anterior (front) and posterior (back) segments of the eye, often requiring specialized ophthalmic attention to prevent discomfort and vision loss.
Ocular Surface Manifestations
The most frequent way Scleroderma affects the eyes is through severe dry eye syndrome, known medically as keratoconjunctivitis sicca (KCS). This condition affects a large percentage of patients, with prevalence reported in various studies to range from approximately 37% to 79%.
The underlying cause is multifactorial, stemming directly from the disease processes. Fibrosis-related impairment of the lacrimal glands significantly reduces the production of the aqueous component of the tear film. Simultaneously, inflammation and dysfunction of the meibomian glands, which produce the tear film’s outer lipid layer, contribute to excessive tear evaporation. This lack of tear film homeostasis leads to characteristic symptoms such as persistent grittiness, redness, burning, and a foreign body sensation.
Untreated or severe surface dryness can have progressive consequences for the eye’s integrity. Chronic lack of lubrication increases the risk of superficial punctate keratopathy, which involves damage to the delicate corneal surface cells. In advanced cases, this can lead to corneal abrasions, filamentary keratitis, and an increased susceptibility to eye infections, all of which compromise the transparency and function of the cornea.
Involvement of Eyelids and Orbital Structures
The generalized fibrosis characteristic of Scleroderma directly impacts the periocular skin, leading to specific mechanical issues with the eyelids. Excessive deposition of collagen causes the eyelid skin to become stiff, tight, and thickened, a manifestation seen in over half of Scleroderma patients. This remodeling restricts the natural mobility and flexibility of the eyelids, which are essential for blinking and tear distribution.
The resulting skin tightness can lead to lagophthalmos, a condition where the eyelids cannot close completely over the eyeball, particularly during sleep. Lagophthalmos prevents the ocular surface from being properly lubricated and protected, exacerbating dry eye symptoms and increasing the risk of exposure-related corneal damage. In some instances, the tightening and scarring of the eyelid skin can also cause the lid margin to turn inward (entropion) or outward (ectropion), leading to further irritation.
While rare, the connective tissue within the orbit, the bony socket that holds the eyeball, can also be affected. Fibrotic changes have been documented in the orbital fat and surrounding tissues, especially in localized forms. These deeper changes can potentially cause restricted eye movement or are associated with conditions like orbital fat atrophy.
Systemic Sclerosis and Vision-Threatening Conditions
The systemic nature of Scleroderma, particularly its effect on blood vessels, creates the potential for serious complications in the posterior segment of the eye. The diffuse vasculopathy of the disease can affect the microcirculatory system of the retina and choroid, the highly vascular layer beneath the retina. Retinal microvascular abnormalities, such as hemorrhages, microaneurysms, and cotton-wool spots, have been observed in a significant portion of Scleroderma patients.
A more immediate threat to vision arises from the link between Scleroderma and malignant hypertension, often occurring during a Scleroderma Renal Crisis (SRC). This rapid, severe spike in blood pressure can trigger acute hypertensive retinopathy. Ocular signs of a hypertensive crisis include flame-shaped retinal hemorrhages, hard exudates, and swelling of the optic nerve head, known as optic disc edema.
In the most severe cases of malignant hypertension linked to SRC, a person may develop exudative retinal detachment, where fluid leaks beneath the retina, causing a sudden and dramatic loss of vision. These posterior segment changes are direct indicators of systemic organ damage and require immediate and aggressive medical intervention to control the underlying blood pressure and prevent permanent vision loss.
Monitoring and Treatment Strategies
Regular, comprehensive eye examinations are a necessary part of managing Scleroderma to monitor both anterior and posterior segment health. Diagnostic tests like the Schirmer’s test or tear break-up time (TBUT) are used to objectively measure tear production and stability for dry eye evaluation. An ophthalmologist will also perform fundoscopy, a direct examination of the retina and optic nerve, to screen for subtle microvascular changes or signs of hypertensive damage.
Treatment for dry eye syndrome typically begins with frequent application of preservative-free artificial tears and lubricants to supplement the deficient tear film. For more persistent cases, prescription eye drops like topical cyclosporine or lifitegrast can be used to manage inflammation on the ocular surface. Punctal plugs, which temporarily block tear drainage, may be considered, although their effectiveness can sometimes be limited by the mechanical tightness of the surrounding skin.
Management of mechanical eyelid issues, such as lagophthalmos, relies on intensive lubrication with thicker nighttime ointments and moisture goggles to protect the cornea during sleep. Vision-threatening conditions, particularly severe hypertensive retinopathy, require urgent coordination between the ophthalmologist and a rheumatologist or nephrologist. Treating the underlying systemic issue, such as stabilizing blood pressure with appropriate antihypertensive therapy, is the primary and most effective way to resolve associated posterior segment complications.