Therapy Insight: The Recognition and Treatment of Retinal Manifestations of Systemic Vasculitis

Table 1.  Features of Arteritic and Nonarteritic Anterior Ischemic Optic Neuropathy.^[38]

Features	Arteritic AION	Nonarteritic AION
Incidence	The RR of GCA is 20:100,000 among people aged ≥50 years, and the RR of arteritic AION in GCA varies from 14% to 70%	2.3–10.2:100,000 people aged ≥50 years
Pathogenesis	Involvement of posterior ciliary artery circulation by a vasculitic process	Decreased perfusion of optic disk Nature and location of vasculopathy is unknown
Risk factors	GCA or polymyalgia rheumatica Other vasculitides are a rare cause of arteritic AION	Age, small optic disk, absent physiologic cup, white ethnicity, hypertension, diabetes mellitus, ischemic heart disease, smoking
Presentation	Loss of visual acuity (more than in nonarteritic AION)	Variable loss of visual acuity
Signs	Dense central scotoma, pale optic disk with diffuse or segmental edema, occasionally BRAO or CWS separate from the disk	Altitudinal visual-field defect, diffuse or segmental disk edema, which is often superior or inferior, small optic disk
ESR	Usually elevated but a normal value does not exclude arteritic AION[39]	Normal
Treatment	Treat the underlying vasculitis with high-dose systemic corticosteroid	Exclude arteritic AION and modify risk factors No established treatment
Prognosis	Visual prognosis poor Without treatment, up to 70% might suffer contralateral AION within 1 week[12]	Better than arteritic AION 50% have visual acuity >20/64 at 6 months Other eye involved in 15% of cases at 5 years

 

Abbreviations: AION, anterior ischemic optic neuropathy; BRAO, branch retinal artery occlusion; CWS, cotton-wool spots; ESR, erythrocyte sedimentation rate; GCA, giant cell arteritis; RR, reported risk.

Table 1.  Features of Arteritic and Nonarteritic Anterior Ischemic Optic Neuropathy.^[38]

Features	Arteritic AION	Nonarteritic AION
Incidence	The RR of GCA is 20:100,000 among people aged ≥50 years, and the RR of arteritic AION in GCA varies from 14% to 70%	2.3–10.2:100,000 people aged ≥50 years
Pathogenesis	Involvement of posterior ciliary artery circulation by a vasculitic process	Decreased perfusion of optic disk Nature and location of vasculopathy is unknown
Risk factors	GCA or polymyalgia rheumatica Other vasculitides are a rare cause of arteritic AION	Age, small optic disk, absent physiologic cup, white ethnicity, hypertension, diabetes mellitus, ischemic heart disease, smoking
Presentation	Loss of visual acuity (more than in nonarteritic AION)	Variable loss of visual acuity
Signs	Dense central scotoma, pale optic disk with diffuse or segmental edema, occasionally BRAO or CWS separate from the disk	Altitudinal visual-field defect, diffuse or segmental disk edema, which is often superior or inferior, small optic disk
ESR	Usually elevated but a normal value does not exclude arteritic AION[39]	Normal
Treatment	Treat the underlying vasculitis with high-dose systemic corticosteroid	Exclude arteritic AION and modify risk factors No established treatment
Prognosis	Visual prognosis poor Without treatment, up to 70% might suffer contralateral AION within 1 week[12]	Better than arteritic AION 50% have visual acuity >20/64 at 6 months Other eye involved in 15% of cases at 5 years

 

Abbreviations: AION, anterior ischemic optic neuropathy; BRAO, branch retinal artery occlusion; CWS, cotton-wool spots; ESR, erythrocyte sedimentation rate; GCA, giant cell arteritis; RR, reported risk.

Table 2.  Uncommon vasculitides and their retinal manifestations

Type of vasculitis	Vessels affected	Retinal featuresa	Other features
Takayasu arteritis	Large elastic arteries, selected muscular arteries[9]	OIS, CWS and rarely AION[40]	OIS can resolve following bypass surgery to improve carotid flow[41]
Goodpasture's disease	Small vessels[1]	Rarely retinal hemorrhages, subretinal neovascularization[42,43]	None defined
Henoch–Schönlein purpura	Arterioles and venules, often small arteries and veins[1]	Rarely BRAO[44]	BRAO occurs in association with cerebral vasculitis[44]
pANCA-associated vasculitis	Small vessels[1]	CWS, AION, posterior scleritis, choroiditis, CRAO[45]	None defined
Scleroderma, including CREST syndrome	Small vessels	Rarely CWS, ischemic retinopathy[46]	Choroidal perfusion abnormalities on angiography (asymptomatic and not clinically observable)[47]
Vasculitis associated with rheumatoid arthritis	Small to medium vessels	Rarely AION retinal vasculitis, choroiditis[48,49,50]	None defined

 

^aAll might have associated hypertensive retinopathy. Abbreviations: AION, anterior ischemic optic neuropathy; BRAO, branch retinal artery occlusion; CRAO, central retinal artery occlusion; CREST, scleroderma characterized by at least two of the following: calcinosis, Raynaud's syndrome, loss of muscular control of the esophagus, sclerodactyly, telangiectasia; CWS, cotton-wool spots; OIS, ocular ischemic syndrome; pANCA, perinuclear antineutrophil cytoplasmic antibody.

Authors and Disclosures

M Stanford holds the Chair of Clinical Ophthalmology at King's College London and practices at the Medical Eye Unit at St Thomas' Hospital, London, UK. P Aristodemou is a Specialist Registrar in Ophthalmology at the Royal United Hospital, Bath, UK.

Competing Interests: The authors declared no competing interests.

Sidebar: Key Points

• The pathology of the systemic vasculitides can involve the blood supply to the eye, causing retinal signs

• The types of posterior segment clinical signs relating to the various vasculitides are influenced by the diameter of vessel involved

• With the exception of giant cell arteritis and hypertensive retinopathy, fundal signs related to systemic vasculitides are uncommon or even rare

• The vasculitic process typically affects the retinal arteries rather than the veins, and these signs are not specific to any type of vasculitis

• Often, the development of retinal signs heralds a relapse of the underlying vasculitis, and urgent referral to a rheumatologist is required

• The possibility that retinal signs might be secondary to iatrogenic immunosuppression should be borne in mind, as rapid and specific treatment for retinal infections might preserve vision

Sidebar: Box 1 Principles of management of retinal features related to systemic vasculitis.

Cotton-wool spots

Cotton-wool spots alone do not require specific treatment, although their presence can indicate recurrence of systemic disease, which might require treatment.

Central or branch retinal artery occlusion

The pathogenesis of vasculitic retinal artery occlusions is related to inflammation of vessel walls. Pathogenesis differs to that of the more common embolic retinal artery occlusion, for which there have been limited reports of restoration of blood flow (although the success rate of such attempts is disappointing). There are no such published cases for arteritic retinal artery occlusion and treatment in such cases is for the underlying pathology, to prevent further vascular occlusions.

Choroidal ischemia

There is no specific treatment for choroidal ischemia, apart from treating the underlying systemic disease.

Anterior ischemic optic neuropathy

See the treatment recommendations for anterior ischemic optic neuropathy secondary to giant cell arteritis. Restoration of vision is uncommon, and treatment is aimed at prevention of an attack in the uninvolved eye. Long-term treatment is for the underlying systemic vasculitis.

Ocular ischemic syndrome

The restoration of arterial perfusion is possible in cases such as Takayasu's arteritis, in which improvement and resolution of the ocular ischemic syndrome is well documented following bypass graft surgery.^[41] In cases of established ocular ischemic syndrome, panretinal photocoagulation is reserved for cases where there is retinal or iris neovascularization, in an attempt to reduce production of angiogenic factors. The response to this treatment is less favorable than in patients with diabetic retinopathy.

Posterior scleritis and optic nerve compression

Optic nerve compression requires urgent treatment. In the case of posterior scleritis, systemic immunosuppression for the underlying systemic disease will reduce inflammation and decompress the optic nerve.

Hypertensive retinopathy

Treatment for systemic hypertension and underlying disease. Retinal signs do not require specific treatment.

Visual symptoms or the discovery of any of the above retinal signs should prompt consultation with an ophthalmologist for advice.