What systemic evaluation is needed when retinal vasculitis is noted?

Systemic Evaluation for Retinal Vasculitis

When retinal vasculitis is identified, the systemic workup should be guided primarily by the patient's age, symptoms, and clinical presentation rather than ordering an exhaustive battery of tests, as most cases are either isolated ocular disease or associated with non-vasculitic systemic conditions—not classical systemic vasculitides. 1

Immediate Priority: Rule Out Giant Cell Arteritis (GCA)

In patients over age 50, GCA must be excluded emergently as it represents a sight-threatening and life-threatening emergency. 2

• Obtain immediately: Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and complete blood count with platelets 2
• Screen for GCA symptoms: Headaches, scalp tenderness, jaw claudication, temporal artery tenderness, malaise, fatigue, fever, diplopia, weight loss, polymyalgia rheumatica, or recent vision loss 2
• If GCA is suspected clinically, initiate systemic corticosteroids immediately before laboratory confirmation to prevent vision loss in the fellow eye 2

History-Directed Evaluation Approach

The diagnostic workup should be symptom-driven rather than reflexive. 3 A comprehensive review of systems is critical to direct appropriate testing. 2

Key Historical Elements to Elicit:

• Embolic disease symptoms: Transient ischemic attacks, lateralizing weakness, paresthesias, amaurosis fugax 2
• Systemic inflammatory symptoms: Fever, weight loss, night sweats, arthralgias, skin rashes 4
• Infectious exposures: Tuberculosis, syphilis, HIV, toxoplasmosis, histoplasmosis 2
• Autoimmune disease features: Joint pain/swelling, oral/genital ulcers (Behçet's), dry eyes/mouth, photosensitivity 4, 5
• Cardiovascular risk factors: Smoking, hypertension, diabetes, hyperlipidemia, atrial fibrillation 2

Age-Stratified Evaluation

Patients Under 50 Years Old:

Workup should focus on vasculitis, hypercoagulability, and inflammatory conditions. 2

• Autoimmune/inflammatory screening: Antinuclear antibody (ANA), anti-double-stranded DNA, complement levels (C3, C4), antineutrophil cytoplasmic antibodies (ANCA) if symptoms suggest systemic vasculitis 4
• Hypercoagulability panel: Anticardiolipin antibodies, lupus anticoagulant, protein C/S, factor V Leiden if thrombotic features present 2
• Infectious workup: Syphilis serology (RPR/VDRL, FTA-ABS), tuberculosis testing (PPD or QuantiFERON), HIV testing 2
• Chest radiography: To screen for sarcoidosis, tuberculosis, or lymphoma 2

Patients Over 50 Years Old:

Workup should prioritize embolic sources and cardiovascular disease. 2

• Urgent stroke evaluation: Refer to stroke center or emergency department within 24 hours for diffusion-weighted MRI, as silent brain infarction occurs in 19-25% of retinal arterial occlusions 2, 6
• Embolic source investigation: Carotid duplex ultrasound, echocardiography (transthoracic or transesophageal), electrocardiogram 2
• Cardiovascular risk assessment: Fasting lipid panel, hemoglobin A1c, blood pressure monitoring 2

Specific Disease Associations to Consider

Retinal vasculitis is rarely caused by classical systemic vasculitides (only 1.4% of cases). 1 More common associations include:

• Behçet's disease: Most common systemic vasculitis causing retinal vasculitis (14 of 207 cases in one series) 1
• Sarcoidosis: Non-vasculitic inflammatory disease (13 of 207 cases) 1
• Systemic lupus erythematosus: Screen with ANA and anti-dsDNA if clinical suspicion 4, 3
• Intraocular infections: Cytomegalovirus, toxoplasmosis, syphilis, tuberculosis (29 of 207 cases) 1
• Isolated retinal vasculitis: Accounts for 5.7% of all retinal vasculitis cases and requires exclusion of systemic disease 5

Critical Pitfalls to Avoid

Do not order an exhaustive panel of tests in asymptomatic patients with negative review of systems. 3 In one study, 20.8% of patients without suggestive symptoms had false-positive results that led to unnecessary investigations, and none developed systemic disease during 4 years of follow-up. 3

Recognize that bilateral retinal phlebitis in young women may represent isolated retinal vasculitis requiring only exclusion of systemic disease rather than extensive immunosuppression initially. 5

In diabetic patients with suspected GCA, monitor glucose closely as systemic corticosteroid treatment will destabilize glycemic control. 2

When Systemic Disease is Not Identified

If initial history-directed evaluation is unrevealing, the patient likely has primary/isolated retinal vasculitis. 5, 3 These patients require:

• Multidisciplinary assessment involving ophthalmology and rheumatology to confirm single-organ disease 5
• Close ophthalmologic follow-up as visual outcomes are often poor despite treatment (only 27% improve) 5
• Periodic reassessment for development of systemic manifestations, though this is uncommon 3