What are the causes of retinal vasculitis, listed from most prevalent to least prevalent?

Causes of Retinal Vasculitis: Most to Least Prevalent

The most common systemic causes of retinal vasculitis are Behçet's disease, sarcoidosis, and systemic lupus erythematosus, with infectious etiologies (particularly toxoplasmosis, HSV, and VZV) also representing major contributors. 1, 2, 3

Most Prevalent Causes

Infectious Etiologies

• Toxoplasmosis is one of the most common parasitic causes of retinal vasculitis, presenting as retinochoroiditis with high-quality evidence 1
• Herpes simplex virus (HSV) produces retinal vasculitis with immunologically mediated infiltrates, supported by high-strength evidence 1
• Varicella-zoster virus (VZV) causes retinal vascular inflammation with high-level evidence 1

Systemic Inflammatory Diseases

• Behçet's disease is the most frequently encountered systemic disease associated with retinal vasculitis, predominantly affecting veins 3, 4, 5
• Sarcoidosis commonly presents with venous involvement and represents a major systemic inflammatory condition, with chronic sarcoidosis showing retinal vasculitis in 37.5% of affected patients 1, 3, 4
• Systemic lupus erythematosus is well-established as causing retinal vasculitis with arterial or mixed arteriovenous involvement, showing a 3.5-fold increased incidence of retinal vascular occlusions 1, 6, 3

Moderately Prevalent Causes

Autoimmune/Rheumatologic Conditions

• Multiple sclerosis is associated with retinal vasculitis, predominantly with venous involvement, though periphlebitis occurs in only 25.1% of MS patients with ocular manifestations 3, 4
• Rheumatoid arthritis can be associated with retinal vascular inflammation with moderate evidence 1
• Granulomatosis with polyangiitis (formerly Wegener's) produces retinal vasculitis as part of systemic manifestations 1, 7

Drug-Induced Vasculitis

• Methamphetamine, intravenous immunoglobulins, opioids, hydralazine, antifibrotics, antibiotics, and leukotrienes can all cause drug-induced retinal vasculitis with moderate evidence 1

Less Common Causes

Systemic Vasculitides

• Polyarteritis nodosa may cause inflammatory retinal vessel involvement 1
• Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) is associated with retinal vasculitis with level II evidence 1
• Microscopic polyangiitis can manifest with retinal vascular inflammation with moderate evidence 1
• Kawasaki disease (primarily pediatric) may involve retinal vessels with high-level evidence 1

Infectious Etiologies (Less Common)

• Epstein-Barr virus may produce retinal vasculitis with low-level evidence 8, 1
• Onchocerca (nematode) may cause retinal vascular inflammation with moderate evidence 1

Rare Causes

• Relapsing polychondritis may produce retinal vasculitis with low-level evidence 1

Critical Emergency Exclusion

Giant cell arteritis (GCA) represents an ophthalmologic emergency that must be excluded promptly in patients over 50 years of age presenting with retinal vascular inflammation, as it can cause central retinal artery occlusion and requires immediate corticosteroid therapy to prevent fellow eye involvement. 8, 1 GCA typically presents with temporal tenderness, jaw claudication, weight loss, elevated ESR/CRP, and optic disc swelling with absence of emboli 8

Clinical Patterns by Vascular Compartment

• Venous predominance is seen with Behçet's disease, sarcoidosis, and multiple sclerosis 3, 4
• Arterial or mixed arteriovenous involvement characterizes systemic lupus erythematosus and necrotizing vasculitides 6, 3
• Periphlebitis (36.5%) is diagnosed far more commonly than periarteritis (4.7%) across all causes 4