Scleroderma can affect nearly every structure of the eye, from the surface tears to the deepest vascular layers of the retina. The most common problem is severe dry eye, found in the majority of people with systemic sclerosis, but the disease can also tighten the eyelids, thin the blood-vessel-rich choroid layer behind the retina, and raise the risk of cataracts and glaucoma. Because scleroderma is rare and its eye involvement often creeps in gradually, these complications are frequently caught late or missed entirely.
Dry Eye Is the Most Common Eye Problem
Dry eye disease is the hallmark ocular complaint in scleroderma. One study found severe dry eye in about 84 percent of patients with systemic sclerosis, and it tended to be worse in women.1PubMed Central. Dry Eye in Systemic Sclerosis Patients: Novel Methods to Monitor Disease Activity A separate study reported dry eye disease in roughly two-thirds of the patients examined.2Ocular Immunology and Inflammation. A Wide Spectrum of Ocular Manifestations Signify Patients with Systemic Sclerosis The numbers vary between studies, but the consistent finding is that dry eye is almost ubiquitous in this population.
What makes scleroderma-related dry eye different from ordinary dry eye is that it has multiple reinforcing causes. The fibrosis that defines scleroderma can damage the meibomian glands in the eyelids, which produce the oily layer of the tear film. In the study mentioned above, meibomian gland disease was present in nearly 87 percent of cases, and the severity of dry eye correlated strongly with skin thickening as measured by clinicians.1PubMed Central. Dry Eye in Systemic Sclerosis Patients: Novel Methods to Monitor Disease Activity In other words, the worse your skin involvement, the worse your tear quality tends to be.
Delayed diagnosis is a real concern. When dry eye occurs alongside a systemic autoimmune disease, it is often written off as a nuisance rather than treated aggressively. Left unmanaged, autoimmune dry eye can progress to corneal ulceration, thinning of the cornea, and even inflammation of deeper eye structures, all of which threaten vision.3European Journal of Ophthalmology. Dry eye disease, a prominent manifestation of systemic autoimmune disorders If you have scleroderma and your eyes feel gritty, burn, or your vision blurs intermittently, it is worth raising with both your rheumatologist and an ophthalmologist, not just managing it with over-the-counter drops.
Eyelid Tightening and Surface Changes
Scleroderma’s hallmark process is fibrosis of the skin and connective tissues, and the eyelids are no exception. In a 2024 review, eyelid and conjunctival abnormalities were described as among the most prevalent ocular findings, affecting up to two-thirds of patients.4PubMed. Ocular Involvement in Systemic Sclerosis: Updated Review and New Insights on Microvascular Impairment The specific problems include:
- Lagophthalmos: the eyelids cannot close completely, leaving a strip of cornea exposed during sleep or blinking.
- Blepharophimosis: the eyelid opening narrows as the skin tightens, sometimes restricting the visual field.
- Telangiectasia: tiny dilated blood vessels appear on the eyelid skin, mirroring the telangiectasias seen on the face and hands.
- General rigidity: the lids lose their normal elasticity, making blinking less effective at spreading tears across the cornea.
The connection between eyelid tightening and dry eye is direct. If your lids cannot close fully, the cornea dries out overnight. If they are too rigid to blink efficiently, the tear film breaks apart between blinks. These mechanical problems compound the glandular damage mentioned earlier, creating a double hit on the ocular surface. People with scleroderma who notice their eyes are worse in the morning, or who wake up with a sensation that the lid is “stuck” to the eye, often have some degree of lagophthalmos.
Damage Deeper In: The Retina and Choroid
Scleroderma is fundamentally a vascular disease as much as it is a fibrotic one, and the eye contains some of the smallest, most vulnerable blood vessels in the body. The choroid, a thin vascular layer sandwiched between the retina and the outer wall of the eye, appears to be particularly affected. One study found that people with scleroderma had significantly thinner choroids in the nasal, temporal, and central subfoveal areas compared to healthy controls, consistent with reduced blood supply causing tissue atrophy.5Eye. Evaluation of choroidal thickness in patients with scleroderma A separate study using advanced retinal imaging confirmed reduced perfusion in both the retinal vascular layers and the deeper choroidal layers known as Sattler’s layer and Haller’s layer.6PubMed Central. Correlation of retinal and choroidal microvascular impairment in systemic sclerosis
These blood flow changes are not always obvious on a standard eye exam. Many patients have no visual symptoms from them, at least early on. But in some cases the vascular damage is visible. Cotton wool spots, which are small white patches on the retina caused by blocked blood flow to the nerve fiber layer, have been reported in scleroderma patients even when they do not have diabetes or high blood pressure, which are the usual culprits.7PubMed Central. A case of systemic sclerosis/lupus overlap syndrome presenting with bilateral cotton wool spots In one clinical study, retinal abnormalities of various kinds were present in about half of patients examined.2Ocular Immunology and Inflammation. A Wide Spectrum of Ocular Manifestations Signify Patients with Systemic Sclerosis
There is also emerging evidence that the retinal nerve fiber layer, the tissue that transmits visual signals to the brain via the optic nerve, may thin over time in scleroderma. Researchers found that patients with systemic sclerosis had a thinner temporal retinal nerve fiber layer compared to controls, and that longer disease duration correlated with more thinning in several retinal zones.8ACR Meeting Abstracts. Structural Abnormalities of the Optic Nerve Head and Retinal Nerve Fiber Layer Using Optical Coherence Tomography in Patients with Systemic Sclerosis Whether this translates into measurable vision loss over a lifetime is not yet clear, but it underscores that scleroderma’s vascular effects reach deep into the eye.
Cataracts, Glaucoma, and Intraocular Pressure
Cataracts and glaucoma show up at surprisingly high rates in scleroderma. One study of 51 patients found cataracts in about half and glaucoma in roughly one in five.2Ocular Immunology and Inflammation. A Wide Spectrum of Ocular Manifestations Signify Patients with Systemic Sclerosis Part of this is likely related to long-term corticosteroid use, which is well known to accelerate cataract formation and raise eye pressure. But scleroderma’s own effects on the eye may also play a role.
Measuring intraocular pressure accurately in scleroderma patients is tricky. The disease changes the biomechanical properties of the cornea, the structure through which standard pressure readings are taken. One study using a specialized device found that scleroderma patients had higher average pressure readings and different corneal resistance than healthy controls.9Cornea. Corneal Hysteresis, Corneal Resistance Factor, and Intraocular Pressure Measurement in Patients with Scleroderma Using the Reichert Ocular Response Analyzer The clinical implication is that a “normal” pressure reading in someone with scleroderma might not be truly normal if the cornea’s stiffness is artificially inflating the measurement. This is important to keep in mind when being screened for glaucoma.
When Scleroderma Affects the Face and Eye Socket
The discussion so far has focused on systemic sclerosis, the form of the disease that affects internal organs and widespread skin. But localized scleroderma, particularly a subtype called en coup de sabre that creates a linear band of fibrosis across the forehead or face, can cause a different and sometimes dramatic set of eye problems. When that fibrotic band crosses the brow or orbital area, it can affect the eye on that side directly.
A study of patients with facial en coup de sabre found a range of serious complications in the eye on the affected side, including blindness from corneal perforation or retinal detachment, restricted eye movement with double vision, irregular astigmatism, and dense cataracts.10PubMed Central. Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre Some patients developed enophthalmos, a sunken appearance of the eye, likely from the fibrotic process shrinking the tissues around the orbit. The researchers also found evidence of smaller eye measurements on the affected side, suggesting a long-term negative effect of overlying skin disease on the eye’s own growth and structure.
In children, the fibrosis can involve the extraocular muscles themselves. One case report described an eight-year-old with linear scleroderma who developed strabismus fixus, a condition in which fibrosis of the medial rectus muscle locked the eye in a fixed inward-looking position. Imaging showed the affected muscles were abnormally bulky, and biopsy confirmed diffuse fibrosis.11PubMed. A case of linear scleroderma “en coup de sabre” with strabismus fixus in a child Similar muscle involvement has been reported in systemic sclerosis, where at least one patient developed ocular myositis causing double vision that resolved with corticosteroid treatment.12JAMA Internal Medicine. Inflammatory Ocular Myopathy in Systemic Sclerosis (Scleroderma): A Case Report and Review of the Literature
Despite the potential severity of these complications, ophthalmologic screening in patients with linear scleroderma of the face is uncommon. A scoping review found that only about 3 percent of reported cases had documented eye screening, far lower than the rates for neurological or dental evaluation.13PubMed Central. Linear Scleroderma of the Head – Updates in management of Parry Romberg Syndrome and En coup de sabre: A rapid scoping review across subspecialties Given the range of problems that can develop, anyone with facial linear scleroderma should have a thorough ophthalmologic evaluation, even if they have no eye symptoms.
Eye Surgery Risks in Scleroderma
If you have scleroderma and need eye surgery, there are a few extra considerations your surgeon should be aware of. Cataract surgery is the most common scenario. One case report documented a patient with systemic sclerosis who experienced nearly identical zonular dehiscence, a tearing of the fine fibers that hold the lens in place, during cataract surgery on each eye, with two different surgical teams on separate occasions. The surgeons also encountered unusually steep corneas and iris tissue that prolapsed more easily than expected, and hypothesized that scleroderma-related zonular instability was the underlying cause.14American Journal of Ophthalmology Case Reports. Bilateral zonular dehiscence during cataract surgery in a patient with systemic sclerosis
Laser vision correction is a different concern. A study looking at excimer laser surgery (LASIK and PRK) in patients with collagen vascular diseases, including scleroderma, found that most complications were similar to what would be expected in people without these conditions. The one exception was a case of peripheral flap melt, an uncommon healing complication, which responded to topical steroids.15PubMed. Outcomes and complications of excimer laser surgery in patients with collagen vascular and other immune-mediated inflammatory diseases Many surgeons still consider autoimmune connective tissue diseases a relative contraindication to LASIK because of the risk of abnormal corneal healing, but the evidence is thinner than most patients realize. It is a conversation worth having with a surgeon experienced in autoimmune patients rather than assuming the answer is automatically no.
The Eye as a Window into Whole-Body Vascular Damage
One of the more interesting developments in scleroderma eye research is the realization that retinal imaging can serve as a noninvasive way to track the same microvascular damage that scleroderma causes throughout the body. The technology involved is called optical coherence tomography angiography, or OCTA, which produces detailed maps of blood flow in the retina without injecting any dye.
Studies using OCTA consistently show that scleroderma patients have reduced vessel density in both the superficial and deep layers of the retinal circulation, as well as around the optic nerve.16European Journal of Internal Medicine. Optical coherence tomography angiography: a window on systemic sclerosis microangiopathy What makes this clinically interesting is that these retinal changes appear to mirror what is happening in the tiny blood vessels elsewhere. Researchers have found that nailfold capillaroscopy, a standard technique where a microscope examines the capillaries at the base of the fingernails, correlates with retinal vessel density in scleroderma patients. Patients with fewer and more damaged nail-fold capillaries also had lower retinal blood flow measurements.17Journal of Rheumatic Diseases. The correlation between retinal microvascular changes by optical coherence tomography angiography and nailfold capillaroscopic findings in patients with systemic sclerosis 18PubMed Central. Retinal Microvasculature in Systemic Sclerosis Patients and the Correlation between Nailfold Capillaroscopic Findings and Optical Coherence Angiography Results
This correlation raises the possibility that a quick retinal scan could someday supplement or even replace more invasive ways of tracking scleroderma’s vascular progression. It is not there yet as a clinical tool for rheumatologists, but the research is moving in that direction, and it represents a genuine reason for scleroderma patients to get regular dilated eye exams even when they feel fine.
Why Symptoms and Test Results Often Disagree
A frustrating quirk of scleroderma-related eye disease is that how your eyes feel does not always match what the tests show. One study evaluating both dry eye symptoms and clinical measurements in scleroderma patients concluded that dry eye symptoms had a moderate impact on vision-related quality of life but did not correlate well with objective clinical findings.19PubMed. Evaluation of dry eye signs and symptoms in patients with systemic sclerosis You might have significant corneal surface damage but relatively mild discomfort, or you might feel miserable while your tear tests look only mildly abnormal.
This disconnect is not unique to scleroderma, but it is pronounced here, likely because the fibrosis and nerve damage that accompany the disease can dull corneal sensation. If the cornea’s own nerves are less sensitive, you may not feel the irritation that would normally signal a problem. A separate cross-sectional study found that lower tear production, as measured by the Schirmer test, did show a weak to moderate link with poorer disease-related quality of life scores, suggesting that at least some of the eye burden does register in how patients rate their overall well-being.20PubMed. The relationship of ocular parameters with clinical parameters and disease-related quality of life in patients with systemic sclerosis: A cross-sectional study
The practical takeaway is that relying on symptoms alone is not enough. Regular eye examinations with objective testing, including tear film assessment, intraocular pressure measurement, and ideally retinal imaging, can catch problems that you would not notice on your own. The retinal vascular changes and nerve fiber thinning discussed earlier can progress silently. For scleroderma patients, an annual comprehensive eye exam is a reasonable baseline, with more frequent visits if the disease is active or if any of the complications above have been identified.