Introduction
Retinal vasculitis is an inflammatory condition that can cause blindness if left untreated. The eye condition may develop independently or in association with connective tissue disease, infection, or malignancy. In rheumatic diseases, the retinal examination is necessary as the vasculitis is asymptomatic in the early stage. Progression of ocular inflammation causes uveitis (inflammation of the middle eye layer), scleritis (inflammation of the sclera), and macular edema (blood leaking to the macula).
What Are the Pathological Features of Retinal Vasculitis?
A prominent pathological feature is the presence of perivascular inflammation and necrosis. Necrosis of the endothelial layer of blood vessels causes obstruction and damage, leading to poor blood flow and ischemia of the eye.
What Are the Rheumatic Conditions Presenting With Retinal Vasculitis?
Systemic Lupus Erythematosus (SLE): The severity of the disease can be determined by the vision loss caused by lupus retinopathy. Exacerbation of systemic SLE causes ocular inflammation. Large vessel occlusions and vaso-occlusive retinopathy are a result of antiphospholipid antibodies. In recent years, the incidence of retinopathy has reduced in SLE due to using steroids. Below are a few vascular abnormalities in SLE
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Microvascular Occlusion: SLE retinopathy causes cotton-wool spots that are classic lesions. The focal areas of ischemia interrupt the axoplasmic flow of nerve fibers of the retina due to the occlusion of small arterioles by inflammatory cell infiltration. Lupus retinopathy is not as extensive as other systemic diseases.
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Arterial Occlusion: It is a less common form of retinopathy with central retinal artery obstruction, causing ischemia and loss of vision. The condition is of rapid onset, with the painless blurring of vision, Marcus Gunn afferent papillary defect, retinal whitening with cherry spot appearance of the fovea (small, flat spot in the center of the retina), and macular ischemia. Young patients have acute vision loss due to central artery obstruction.
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Venous Occlusion: The condition is rare and causes permanent eye damage. Arterial occlusion causes venous engorgement, causing central vein occlusion. Retinal and papillary hemorrhage occurs when venous endothelium is damaged.
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Severe ischemia results in retinal neovascularization. The complications of neovascularization, such as tractional retinal detachment and vitreous hemorrhage, cause vision loss.
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Secondary hypertension develops due to renal involvement. The ocular changes due to hypertension include arterial narrowing, intraretinal hemorrhage, hard exudate formation, and hypertensive papilledema.
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Lupus choroidopathy is commonly seen with systemic involvement. Immune complexes are deposited in the choroidal capillaries of SLE.
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The vascular lesion in the eye indicates changes in vasculature in other body parts. The retinal vessel wall and nerve fibers vasculature share the same pathway and exhibit changes simultaneously.
Rheumatoid Arthritis: A small percentage of patients with rheumatoid arthritis exhibit ocular changes. The ophthalmic changes are scleritis, inflammation, and thinning of the peripheral cornea that progress to perforation, ultimately causing blindness. In addition, symptoms of foreign body sensation, presence or absence of eye pain, tearing, photophobia (eye discomfort in bright light), and reduced visual acuity (ability to differentiate shapes) are seen. The ocular changes are manifestations of systemic disease progression and can cause mortality without treatment.
Sarcoidosis: Ocular changes occur in half of the patients. The anterior segment symptoms are granulomatous uveitis, and the posterior segment includes vitritis with or without inflammatory ‘snowballs,’ pre-retinal nodules. Choroidal lesions may also occur. Retinal involvement in the form of peri-phlebitis is a prominent feature of sarcoidosis. Arteries are rarely involved, and discontinuous vessel involvement gives a skip lesion appearance. Yellow perivenous exudate (candle wax dripping) is indicative of sarcoidosis. The condition resolves with corticosteroid treatment.
Adamantiades- Bechet's Disease: It is a multisystem vasculitis affecting arteries and veins and is of unknown cause. Most patients exhibit ocular involvement with a recurrent and explosive exacerbation of intraocular inflammation. Posterior uveitis and panuveitis (diffuse uveitis) with destructive retinal vasculitis are common. Severe and recurrent uveitis causes irreversible retinal damage and blindness. The condition is treated with systemic corticosteroids and immunosuppressive agents, but there is an increased possibility of relapse even with treatment.
Wegner’s Granulomatosis: Many patients exhibit ocular symptoms. 15 % patients present with ocular complaints as early symptoms in early-stage Wegner’s granulomatosis. The symptoms are orbital granuloma, peripheral ulcerative keratitis, corneal granuloma, necrotizing scleritis, and uveitis. Retinal hemorrhages in the peripheral and posterior regions are common.
Hla- B27- Associated Uveitis and Spondyloarthropathies: Uveitis is commonly seen, and the inflammation is acute and recurrent. Anterior segments are commonly involved. However, a few cases with posterior segment involvement, such as vitritis, retinal vasculitis, or macular edema, may be present. The patients may eventually experience blindness.
What Are the Signs and Symptoms of Retinal Vasculitis in Rheumatic Disease?
Symptoms
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The condition is asymptomatic in early stages or vascular changes in the peripheral retina.
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Ischemia causes blurred vision or metamorphopsia (a grid of straight lines appears wavy).
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Reduced vision.
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Reading near is hampered.
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Distance vision is enhanced in near-sighted patients.
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Floaters and ischemia cause visual field deficits.
Signs
Fundoscopy can reveal various inflammatory signs.
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Cotton-wool spots.
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Dots or flame-shaped structures due to retinal hemorrhage.
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Macular edema.
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Preretinal hemorrhage.
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Microaneurysms (disturbances in the diameter of blood vessels to the retina).
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Narrowing of retinal vasculature is limited.
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Arterial occlusion with focal (intermittent) deposits.
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Central or retinal arterial occlusion that appears as cherry red spots.
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Central or retinal venous obstruction.
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Neovascularization of retina.
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Vitreous hemorrhage.
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Anterior segment ischemia.
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Hemorrhagic glaucoma.
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Hypertensive changes (optic disk edema, arteriolar narrowing, hard exudates).
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Optic disk neuritis (optic disk nerve inflammation).
How to Diagnose Retinal Vasculitis in Rheumatic Disease?
Clinical Ophthalmic Examination.
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A detailed medical history includes past medical conditions and details of the presenting illness with ocular and systemic symptoms.
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Doctors must document a history of infections, tumors, drug sensitivity, and rheumatological conditions.
Fluorescein Angiography: Doctors must examine it before administering systemic therapy. Arterial or venous vessels or both may be involved. Retinal vessel sheathing is the main diagnostic sign. Fluorescein angiography helps understand the extent of retinal damage using intravenous dye fluorescein. The active retinal disease shows leakage of dye, causing retinal wall hyperfluorescence.
Optical Coherence Tomography: Utilizes noninvasive interferometry. Cross-sectional full-thickness retinal scans help observe macular edema or determine the cause of poor vision.
How Is Retinal Vasculitis in Rheumatoid Disease Treated?
The severity and extent of the disease decide treatment. The mainstay of treatment is to halt the disease progression and prevent relapse. Patients with retinal vasculitis require systemic treatment to control ocular manifestations.
Medical Therapy
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Corticosteroids: Steroids can be applied topically or taken orally as injections or as intravenous pulse therapy. Treating retinal vasculitis involves systemic administration of steroids adjunct to immunomodulatory agents. Periocular steroid delivery is given if macular edema is present in one eye. Vaso-occlusive disease with antiphospholipid antibodies requires photocoagulation as an adjunct therapy.
Immunomodulators: Immunomodulators are used in poor response of retinal vasculitis to other treatment modes. The commonly used agents are antimetabolites Methotrexate), inhibitors (Cyclosporine), and alkylating agents (Cyclophosphamide). Intravenous administration of immunomodulators has successfully caused remission of retinal vasculitis.
Conclusion
Early diagnosis with adequate treatment can cause remission of the disease and prevent the risk of vision loss. The ocular manifestation must be treated with systemic medication using anti-inflammatory drugs and immunosuppressants. Doctors cannot cure the condition completely, but symptoms are managed with treatment to improve the patient's quality of life.
