Is antineutrophil cytoplasmic antibody (ANCA) associated with scleritis or retinal involvement?

ANCA is Associated with Scleritis

ANCA is strongly associated with scleritis, not retinal involvement. According to the most recent KDIGO 2024 guidelines, ocular involvement is explicitly listed among the commonly affected systems in ANCA-associated vasculitis (AAV), with scleritis being a well-recognized extrarenal manifestation 1.

Primary Ocular Manifestation: Scleritis

The evidence consistently demonstrates that scleritis and episcleritis are the predominant ocular manifestations in ANCA-associated vasculitis:

• Scleritis occurs in up to 87% of AAV patients with ocular involvement 2
• Episcleritis is present in approximately 54.5% of cases 3
• Both anterior scleritis and the more severe necrotizing scleritis are well-documented complications 4, 5, 6

The 2020 European Position Paper on Rhinosinusitis confirms that GPA (granulomatosis with polyangiitis) is strongly associated with ANCA, particularly in patients with widespread disease manifestations including ocular involvement 7.

Retinal Involvement: Less Common but Possible

While scleritis is the hallmark ocular finding, retinal manifestations do occur but are significantly less frequent:

• Retinal vasculitis, exudates, and hemorrhage were present in only 15.2% of AAV patients with ocular disease 2
• Retinal vein occlusion and optic neuropathy have been reported in p-ANCA (MPO-ANCA) positive patients 8
• Combined retinal and choroidal detachment represents a rare but potentially blinding complication 9

Clinical Algorithm for Recognition

When evaluating ANCA-positive patients for ocular involvement, prioritize assessment for:

1. Scleritis/episcleritis (most common - check for ocular pain, redness, nodular scleral lesions)
2. Peripheral ulcerative keratitis (second most common anterior segment finding)
3. Uveitis (present in ~24% of cases, associated with higher disease activity) 2
4. Posterior segment complications (less frequent - retinal vasculitis, optic neuropathy)

Important Clinical Caveats

• PR3-ANCA (c-ANCA) is more strongly associated with ocular manifestations than MPO-ANCA, with 81.8% of patients with ocular involvement being PR3-ANCA positive 3
• Necrotizing scleritis can lead to globe perforation and vision loss if not treated aggressively with systemic immunosuppression 5, 6
• The presence of scleritis in an ANCA-positive patient may indicate active systemic vasculitis requiring intensification of therapy 4
• All AAV patients should undergo structured ophthalmologic examination as ocular involvement can develop at any time during the disease course 1

Rituximab combined with glucocorticoids has proven effective for maintaining remission in ANCA-associated scleritis, particularly in cases refractory to initial therapy 4, 6.