Classification of Retinal Vasculitides Based on Vessels Affected
Retinal vasculitides are classified into three primary categories based on the predominant vessel type involved: arterial vasculitis (retinal arteritis), venous vasculitis (retinal phlebitis), and capillary vasculitis. 1, 2, 3
Primary Classification Framework
The classification system for retinal vasculitis is fundamentally organized by vessel caliber and location:
Venous Vasculitis (Retinal Phlebitis)
- Predominantly affects retinal veins and presents with venous sheathing, perivascular infiltrates, and inflammatory changes along the vessel wall 1
- May be associated with branch retinal vein occlusion, though this should be distinguished from primary thrombotic/embolic vein occlusions which lack true inflammatory vessel wall changes 4
- Venous involvement is more commonly seen in noninfectious etiologies and typically shows less ischemia than arterial disease 5
Arterial Vasculitis (Retinal Arteritis)
- Involves retinal arterioles with arterial sheathing, narrowing, and occlusion 2, 3
- More frequently associated with infectious etiologies and tends to exhibit more severe ischemic changes 6
- Arterial occlusion patterns suggest infectious causes, particularly when combined with retinal or choroidal involvement 6
Capillary Vasculitis
- Affects the retinal capillary network and is predominantly associated with noninfectious retinal vasculitis 6
- Presents with capillary leakage, microaneurysms, and diffuse retinal edema 3
- More difficult to visualize on clinical examination but well-demonstrated on fluorescein angiography 3
Secondary Classification Considerations
Occlusive vs. Non-Occlusive Pattern
- Retinal vasculitis can be further subclassified as occlusive (with vessel closure and ischemia) or non-occlusive (with inflammation but preserved perfusion) 5
- Occlusive disease carries higher risk for complications including neovascularization, vitreous hemorrhage, and neovascular glaucoma 5
- Infectious vasculitis tends toward occlusive patterns with ischemic areas, while noninfectious disease more commonly shows non-occlusive capillary involvement 6
Location-Based Classification
- Peripheral retinal vasculitis affects vessels in the retinal periphery 2
- Posterior pole vasculitis involves vessels near the optic disc and macula 2
- Ultra-widefield imaging has improved detection of peripheral vascular involvement that may be missed on standard examination 5
Important Distinction: Choriovasculitis
- Choriovasculitis is a separate entity involving inflammation of the choroidal circulation posterior to the retina, distinct from retinal vasculitis 4
- Seen in conditions like Vogt-Koyanagi-Harada disease and white dot syndromes 4
- Requires indocyanine green angiography (ICGA) for optimal detection, as fluorescein angiography primarily images retinal vessels 4
Clinical Pitfalls
- Do not confuse retinal vein occlusion with venous vasculitis: retinal vein occlusions are primarily thrombotic/embolic processes lacking the inflammatory vessel wall changes characteristic of true vasculitis 4
- Infectious vasculitis typically shows arterial or venous sheathing/occlusion with ischemic areas, while noninfectious disease more commonly presents with capillary vasculitis 6
- The distinction between vessel types affected has direct therapeutic implications, as infectious and noninfectious etiologies require fundamentally different treatment approaches 7