Case Study

A 29-year-old woman with a 3-year history of systemic lupus erythematosus (SLE) presented with sudden-onset blurry vision in her left eye, accompanied by mild ocular discomfort and increased photosensitivity.

Ocular Complications of Systemic Lupus Erythematosus

She had been non-compliant with her hydroxychloroquine treatment for the past two months due to gastrointestinal side effects.

Ophthalmic examination revealed cotton-wool spots, microaneurysms, and scattered intraretinal hemorrhages along the posterior pole, with perivascular sheathing more pronounced in the superior arcade.

OCT demonstrated inner retinal ischemia with paracentral acute middle maculopathy-like changes. Fluorescein angiography confirmed areas of capillary non-perfusion, vascular leakage, and delayed arteriovenous transit time.

Laboratory testing showed elevated anti-dsDNA, low complement levels, and increased ESR, indicating active systemic disease.

She was immediately started on high-dose systemic corticosteroids and consulted rheumatology for immunosuppressive escalation.

Despite treatment, the affected eye recovered only to 20/60 due to persistent macular ischemia, highlighting the aggressive nature of SLE-associated retinal vasculopathy.

Disease Entity

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder affecting multiple organ systems through immune complex deposition and complement-mediated inflammation.

Ocular involvement occurs in 20–34% of patients and can affect virtually any structure of the eye, from the adnexa to the retina and optic nerve.

Retinal and choroidal manifestations are among the most vision-threatening complications, often correlating strongly with systemic disease activity.

Ocular complications range from mild keratoconjunctivitis sicca to severe retinal vaso-occlusive disease and lupus choroidopathy, making ophthalmologists integral to early detection of systemic flares and guiding timely treatment.

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Pathophysiology

The main pathogenic mechanism in SLE ocular disease is immune complex deposition within small vessels of the eye, leading to:

  • Complement activation and inflammation

  • Endothelial damage and capillary occlusion

  • Vascular leakage and ischemia

  • Breakdown of the blood–retinal barrier

The retina is particularly vulnerable due to its dense microvascular network.
Other mechanisms include:

  • Autoantibody-mediated vascular injury

  • Procoagulant state leading to arterial or venous occlusion

  • Choroidal ischemia secondary to complement activation

  • Drug-induced toxicity (notably hydroxychloroquine)

Epidemiology

  • Ocular involvement occurs in up to one-third of SLE patients.

  • Retinal involvement is reported in 3–15%, typically during active systemic flares.

  • Severe vaso-occlusive retinopathy is more common in patients with antiphospholipid syndrome (APS) or high disease activity.

  • Lupus choroidopathy is strongly associated with renal involvement and hypertension.

Clinical Features

Ocular complications of SLE involve both anterior and posterior segment structures.

1. Anterior Segment Manifestations:

  • Keratoconjunctivitis sicca (most common)

  • Episcleritis or scleritis

  • Peripheral ulcerative keratitis

These are usually associated with systemic disease activity.

2. Retinal Manifestations (Most Vision-Threatening):

Lupus Retinopathy:

  • Cotton-wool spots

  • Intraretinal hemorrhages

  • Microaneurysms

  • Retinal vascular leakage

  • Venous dilation and tortuosity

  • Perivascular sheathing (“vasculitis”)

Severe Vaso-Occlusive Lupus Retinopathy:

  • Branch or central retinal vein occlusion

  • Arterial occlusions

  • Purtscher-like retinopathy

  • Wide areas of capillary non-perfusion

  • Acute macular ischemia

  • Neovascularization and vitreous hemorrhage in late stages

3. Choroidal Manifestations:

  • Lupus choroidopathy

  • Serous retinal detachments due to choroidal ischemia

  • RPE folds and mottling

4. Optic Nerve Involvement:

  • Optic neuritis

  • Ischemic optic neuropathy

  • Papilledema associated with CNS involvement

Examination Findings

  • OCT: inner retinal thinning, ischemic changes, serous retinal detachment in choroidopathy.

  • FA: delayed filling, leakage, non-perfusion, arterial attenuation.

  • ICG angiography: choroidal hypoperfusion and multifocal ischemic spots.

  • Visual fields: arcuate or paracentral defects depending on ischemia.

Ocular Complications of Systemic Lupus Erythematosus

Differential Diagnosis

  • Diabetic retinopathy

  • Hypertensive retinopathy

  • Infectious retinal vasculitis (TB, syphilis, toxoplasmosis)

  • Retinal vasculitis of other autoimmune diseases

  • Antiphospholipid syndrome retinopathy

  • Hydroxychloroquine retinal toxicity (distinct pattern)

Diagnosis

Diagnosis is based on clinical examination and diagnostic imaging combined with systemic assessments.

Essential Investigations:

  • Fluorescein angiography (FA)

  • OCT and OCT-A

  • ICG for choroidal involvement

  • Autoimmune panel: ANA, dsDNA, SSA/SSB

  • Complement levels (C3, C4)

  • APS testing (anticardiolipin, lupus anticoagulant, β2 glycoprotein)

Management

Treatment targets both ocular inflammation and systemic disease.

1. Systemic Immunosuppression

Mainstay of treatment.

  • High-dose corticosteroids (IV methylprednisolone in severe disease)

  • Immunosuppressants:

    • Mycophenolate mofetil

    • Azathioprine

    • Cyclophosphamide (severe vasculopathy or choroidopathy)

    • Methotrexate

2. Biologic Therapy

Used in refractory disease:

  • Belimumab

  • Rituximab (especially in vaso-occlusive retinopathy)

3. Anticoagulation

Required if APS is present.

  • Warfarin or DOACs

  • Aspirin may be used adjunctively

4. Ocular-Specific Management

  • Panretinal photocoagulation for ischemia-induced neovascularization

  • Anti-VEGF injections for macular edema

  • Surgical intervention for complications such as vitreous hemorrhage or tractional detachment

5. Management of Dry Eye

  • Artificial tears

  • Topical cyclosporine or lifitegrast

Prognosis

  • Mild lupus retinopathy often improves with systemic control.

  • Severe vaso-occlusive disease carries a poor prognosis, especially with macular ischemia.

  • Lupus choroidopathy prognosis improves with rapid treatment but may be associated with progression of systemic renal disease.

  • Early diagnosis and aggressive systemic therapy are critical in preventing permanent vision loss.

Ocular Complications of Systemic Lupus Erythematosus

Prevention & Follow-Up

  • Regular ophthalmic evaluation for all SLE patients

  • More frequent monitoring during disease flares

  • Screening for hydroxychloroquine toxicity

  • Close communication with rheumatology

  • Prompt evaluation of any new visual symptoms

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References

  1. Jabs DA. Ocular manifestations of systemic lupus erythematosus. Curr Opin Ophthalmol.

  2. Rangaswamy R, et al. Retinal vascular involvement in SLE. Lupus Science & Medicine.

  3. Nguyen QD, et al. Lupus choroidopathy and vaso-occlusive retinopathy. Ophthalmology.

  4. Arevalo JF, et al. Severe retinal vaso-occlusive disease in SLE: Diagnosis and management. Retina Journal.

  5. Yeh S. Autoimmune retinopathies and systemic autoimmune disease.

Test yourself

A few questions straight from this article.

1 / 10 0 correct

  1. In what proportion of patients with systemic lupus erythematosus does ocular involvement occur?