From the Guidelines
Autoimmune diseases can manifest in the cornea through various conditions, including dry eye syndrome, scleritis, sterile keratitis, and uveitis, particularly in patients with Sjögren's syndrome, rheumatoid arthritis, systemic lupus erythematosus, and other connective tissue disorders. The most recent and highest quality study, 1, highlights the importance of diagnosing and managing Sjögren's syndrome, which is characterized by infiltration of the lacrimal and salivary glands with lymphocytes, leading to dry eye and dry mouth associated with systemic immune dysfunction.
Key Corneal Manifestations
- Dry eye syndrome
- Scleritis
- Sterile keratitis
- Uveitis
- Corneal melting
Diagnosis and Management
According to 1, diagnosis and treatment of underlying systemic immune disorders may decrease morbidity and may even be lifesaving. Patients with dry eye syndrome associated with Sjögren's syndrome may develop other ocular manifestations of immune dysfunction. The latest classification criteria for Sjögren's syndrome are based on the weighted sum of five items, including anti-SSA/Ro antibody positivity, focal lymphocytic sialadenitis, abnormal ocular staining score, Schirmer test result, and unstimulated salivary flow rate.
Treatment Options
Treatment options for dry eye syndrome associated with Sjögren's syndrome include:
- Topical lubricants
- Topical anti-inflammatory therapy
- Tear-conserving strategies
- Oral secretagogues, such as pilocarpine and cevimeline, which have been shown to be effective in treating sicca features, particularly oral dryness 1
- Cyclosporine, which has been found to be beneficial for moderate or severe dry eye 1
Early diagnosis and aggressive management are crucial to prevent permanent vision loss from corneal scarring or perforation, as corneal involvement often parallels systemic disease activity. Regular monitoring of both ocular manifestations and systemic disease activity is essential.
From the FDA Drug Label
The development of secondary ocular infection (bacterial, fungal, and viral) has occurred Fungal and viral infections of the cornea are particularly prone to develop coincidentally with long-term applications of steroids. Keratitis, conjunctivitis, corneal ulcers, mydriasis, conjunctival hyperemia, loss of accommodation and ptosis have occasionally been reported following local use of corticosteroids.
The corneal manifestations of autoimmune diseases are not directly addressed in the provided drug label. However, the label does mention corneal ulcers as an adverse reaction, which can be associated with autoimmune diseases.
- Keratitis and corneal ulcers are mentioned as possible adverse reactions, but the label does not explicitly state that they are corneal manifestations of autoimmune diseases. The FDA label does not provide sufficient information to determine the corneal manifestations of autoimmune diseases 2.
From the Research
Corneal Manifestations of Autoimmune Diseases
The cornea can be affected by various autoimmune diseases, leading to a range of manifestations. Some of the corneal manifestations of autoimmune diseases include:
- Corneal ulceration and melting, which can occur in patients with rheumatoid arthritis 3
- Dry eye disease, which is a common feature of Sjögren's syndrome, lupus, and rheumatoid arthritis 4, 5
- Keratoconjunctivitis sicca, which is a painful condition that can predispose patients to ocular infections 4
- Corneal perforation, which can occur in patients with undiagnosed Sjögren's syndrome 6
- Alterations in corneal biomechanics, which can underlie early stages of autoimmune-mediated dry eye disease 5
Autoimmune Diseases Affecting the Cornea
Several autoimmune diseases can affect the cornea, including:
- Sjögren's syndrome, which is a systemic autoimmune disease characterized by dysfunction of the lacrimal and salivary glands 4, 6
- Rheumatoid arthritis, which is an autoimmune disease that primarily affects the joints but can also affect the cornea 3
- Lupus, which is a systemic autoimmune disease that can affect multiple organs, including the cornea 5
- Mucous membrane pemphigoid, which is an autoimmune disease that can cause corneal inflammation and scarring 7
Treatment of Corneal Manifestations
The treatment of corneal manifestations of autoimmune diseases often involves a multidisciplinary approach, including:
- Topical ophthalmic management, such as artificial tears and corticosteroids 4, 6
- Systemic immunosuppression, such as disease-modifying antirheumatic drugs (DMARDs) and biologic agents 7, 3
- Regenerating agent eye drops, which can aid in tissue regeneration and favorable visual rehabilitation 6
- Autologous serum eye drops, which can provide nutrients and growth factors to the cornea 6