Vasculitis is the inflammation of blood vessel walls throughout the body. When this process affects the vessels within the eye, it is termed ocular vasculitis. The inflammation causes vessel walls to thicken, which restricts blood flow and oxygen delivery to sensitive structures inside the eye, such as the retina and the optic nerve. Any compromise to the blood supply can quickly lead to damage. Ocular vasculitis often serves as a manifestation of a larger, underlying systemic disease, requiring rapid diagnosis and intervention to prevent irreversible visual impairment.
Recognizing the Signs of Ocular Vasculitis
The signs of ocular vasculitis can range from subtle to severe, often presenting as a painless loss of vision that can be sudden or gradual. Patients frequently report seeing floaters, which are dark, drifting spots caused by inflammatory cells migrating into the vitreous gel. Other common symptoms include blurred vision, eye pain, and increased sensitivity to bright light (photophobia).
Symptoms depend on which part of the eye is affected. When retinal vessels are involved (retinal vasculitis), this can lead to visual distortions or the formation of blind spots. Retinal inflammation can cause blood vessel sheathing, where inflammatory cells coat the vessels, making them appear like frosted tree branches.
If inflammation targets the arteries supplying the optic nerve, optic neuropathy can occur, resulting in sudden vision loss. Vasculitis can also affect the protective outer layers of the eye, causing redness and irritation in the episclera or sclera. Damage to the nerves controlling eye movement can lead to double vision.
Identifying the Underlying Cause
Diagnosis begins with a detailed eye examination, where a specialist uses a magnified view to observe signs of inflammation, such as perivascular infiltrates or hemorrhage. To confirm the diagnosis and determine the extent of the disease, physicians utilize advanced imaging techniques. Fluorescein angiography involves injecting a dye into the bloodstream, allowing doctors to visualize blockages, leakage, or areas of non-perfusion in the retinal vessels.
Investigation focuses on identifying the root cause, as ocular vasculitis is frequently secondary to an infectious or non-infectious systemic disease. Blood tests look for general signs of inflammation, measuring markers like C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Specific antibody tests, such as the antineutrophil cytoplasmic antibody (ANCA) test, help diagnose certain types of vasculitis, including Granulomatosis with Polyangiitis.
The workup may include testing for infectious agents like syphilis, tuberculosis, and Lyme disease, as these can trigger retinal vasculitis. Systemic inflammatory conditions strongly associated with ocular involvement include Giant Cell Arteritis (GCA), Behçet’s disease, and Systemic Lupus Erythematosus (SLE). In rare instances, particularly for GCA, a temporal artery biopsy may be performed for microscopic confirmation of vessel wall inflammation.
Medical Management and Treatment Goals
The goal of treatment is to rapidly suppress inflammation to prevent permanent damage to the delicate visual structures. Systemic corticosteroids are the first line of defense. Patients with severe disease or early visual symptoms may be started on high-dose intravenous methylprednisolone, followed by a transition to high-dose oral prednisolone.
While corticosteroids are highly effective for acute inflammation, they are typically tapered after initial disease control to minimize long-term side effects. Immunosuppressive agents, also known as Disease-Modifying Anti-Rheumatic Drugs (DMARDs), are then introduced to achieve durable, steroid-free remission. Common antimetabolites used as steroid-sparing agents include methotrexate, azathioprine, and mycophenolate mofetil.
For patients whose condition is severe, recurrent, or unresponsive, treatment may escalate to biologic agents. TNF-alpha inhibitors like adalimumab and infliximab have shown success, particularly in Behçet’s disease-related retinal vasculitis. Complications like macular edema or the growth of fragile new blood vessels (neovascularization) are managed with local treatments, such as anti-VEGF injections into the eye or retinal laser photocoagulation.
Long-Term Outlook and Follow-Up Care
Ocular vasculitis is often a chronic condition, even when symptoms resolve, necessitating continuous monitoring to manage the underlying systemic disease and prevent relapse. The long-term outlook depends on the extent of damage to the optic nerve and macula before treatment began. If significant retinal ischemia occurred early on, the resulting vision loss may be irreversible.
Follow-up care involves a collaborative approach between an ophthalmologist, who monitors eye inflammation, and a rheumatologist, who manages the systemic disease. Regular eye examinations are required to detect subclinical signs of inflammation or complications like neovascularization. Medication adherence is paramount, as relapses are common.
Even in cases where the vasculitis initially appears confined to the eye, a systemic disease may manifest later. Patients must maintain their follow-up schedule and report any new symptoms, whether ocular or systemic, to their medical team. Lifestyle adjustments, such as avoiding smoking, contribute to the overall management of systemic inflammatory diseases.