Scleroderma is a chronic autoimmune disease characterized by excessive collagen deposition in the skin and connective tissues, leading to fibrosis and vascular abnormalities. This condition can also affect skin, joints, and various organs, including the eyes. When scleroderma affects the eyes, it can cause a range of complications that affect vision and ocular health significantly. This article delves into the specific ways scleroderma impacts the eyes, detailing symptoms, diagnosis, management strategies, and the importance of early intervention.

Scleroderma can involve both the external and internal structures of the eye, leading to various complications:

• Dry Eyes: Decreased tear production due to inflammation of the lacrimal glands is a common ocular manifestation. This can lead to chronic dryness, irritation, and a gritty sensation in the eyes.
• Scleromalacia: Scleroderma may cause thinning and weakening of the sclera, the tough, white outer layer of the eye. This can make the sclera more prone to damage and increase the risk of complications like scleral thinning or perforation.
• Conjunctival Telangiectasia: Characterized by dilated blood vessels in the conjunctiva, which is the thin, transparent membrane covering the white part of the eye and the inner surface of the eyelids. These blood vessels can appear as red or violet lines and are a distinctive feature of ocular involvement in scleroderma.
• Keratoconjunctivitis Sicca: Also known as dry eye syndrome, this condition involves chronic inflammation of the cornea and conjunctiva due to insufficient tear production. It can lead to blurred vision, sensitivity to light (photophobia), and discomfort.
• Retinopathy and Optic Neuropathy: In scleroderma, vascular changes can affect blood flow to the retina, leading to retinopathy. Symptoms may include blurred vision, visual field defects, and, in severe cases, vision loss. Early detection through regular eye exams is crucial to monitor for retinal changes and initiate appropriate treatment to preserve vision. Additionally, scleroderma-related inflammation can rarely extend to the optic nerve, causing optic neuropathy. This condition requires immediate medical attention and may involve systemic immunosuppressive therapy to manage inflammation and protect optic nerve function.
• Cataracts and Glaucoma: Long-term use of corticosteroids to manage systemic symptoms of scleroderma can increase the risk of developing cataracts and glaucoma. Cataracts cause clouding of the lens, leading to impaired vision, while glaucoma results from increased pressure within the eye, damaging the optic nerve and potentially causing irreversible vision loss if left untreated. Regular screening for these conditions is essential, and surgical intervention may be necessary to remove cataracts or manage intraocular pressure in glaucoma.

The symptoms of scleroderma affecting the eyes can vary widely depending on the severity and specific manifestations:

• Dryness and Irritation: Persistent dryness, itching, burning sensation, and foreign body sensation in the eyes.
• Redness: Conjunctival redness due to the presence of dilated blood vessels.
• Blurry Vision: Corneal involvement can cause visual disturbances, such as blurred or distorted vision.
• Photophobia: Increased sensitivity to light, making it uncomfortable to be in brightly lit environments.
• Scleral Changes: Thinning or discoloration of the sclera, which may become more visible due to scleroderma-related changes.

Diagnosing ocular manifestations of scleroderma requires a thorough evaluation by an ophthalmologist familiar with autoimmune conditions:

• Comprehensive Eye Examination: This includes testing visual acuity, examining the external structures of the eye, assessing the conjunctiva and cornea for signs of inflammation or vascular changes, and checking intraocular pressure.
• Schirmer’s Test: A diagnostic test to measure tear production and assess for dry eye syndrome.
• Slit-Lamp Examination: Provides a detailed view of the anterior segment of the eye, allowing the ophthalmologist to examine the cornea, conjunctiva, iris, and lens for abnormalities.
• Ocular Coherence Tomography (OCT): An imaging technique used to visualize and measure the thickness of the retina and cornea, helping to detect structural changes and monitor disease progression.

Managing ocular complications in scleroderma focuses on symptom relief, preventing progression, and preserving vision:

• Artificial Tears and Lubricants: Prescribed to alleviate dryness and maintain ocular moisture.
• Anti-inflammatory Medications: Steroid eye drops or NSAIDs may be used to reduce inflammation in the eyes.
• Immunosuppressive Therapy: In severe cases, systemic immunosuppressants such as methotrexate or cyclophosphamide may be prescribed to control autoimmune activity affecting the eyes.
• Scleral Lenses: Specialized contact lenses designed to improve visual acuity and protect the cornea in cases of severe dryness or irregular corneal shape.
• Surgical Interventions: Rarely, surgical procedures may be necessary to manage complications like scleral thinning, corneal ulcers, or severe conjunctival changes.

Living with scleroderma affecting the eyes requires ongoing self-care and monitoring:

• Eye Protection: Wearing sunglasses with UV protection and avoiding exposure to environmental irritants to minimize dryness and photophobia.
• Hydration: Maintaining adequate hydration by drinking plenty of fluids to support tear production and prevent dry eye symptoms.
• Regular Eye Exams: Routine visits to an ophthalmologist for monitoring, early detection of complications, and adjustments to treatment plans as needed.
• Patient Education: Understanding the symptoms, progression, and management strategies of ocular involvement in scleroderma empowers individuals to actively participate in their eye health care.

The prognosis for ocular involvement in scleroderma varies depending on the severity of systemic disease and timely intervention. With early diagnosis and appropriate management, many ocular symptoms can be effectively controlled, minimizing the risk of vision loss and improving quality of life for patients.

Research into the pathogenesis of scleroderma-related ocular manifestations continues to evolve, focusing on better understanding disease mechanisms and developing targeted therapies. Collaborative efforts among researchers and clinicians aim to improve diagnostic accuracy, treatment efficacy, and long-term outcomes for affected individuals.

Ocular manifestations of scleroderma are varied and can significantly impact vision and quality of life. Early detection, regular eye exams, and a multidisciplinary approach involving rheumatologists and ophthalmologists are essential for effectively managing these complications. By raising awareness and providing specialized care, individuals with scleroderma can mitigate the impact of ocular involvement, preserve their vision, and improve their overall quality of life.

World Eye Care Foundation’s eyecare.live brings you the latest information from various industry sources and experts in eye health and vision care. Please consult with your eye care provider for more general information and specific eye conditions. We do not provide any medical advice, suggestions or recommendations in any health conditions.