Systemic Lupus Erythematosus and the eye
SLE is a chronic, multisystem, autoimmune disease where autoantibodies are produced against autoantigens. There is widespread tissue damage and vasculitis, butterfly facial rash, arthritis, anaemia and renal and cardiovascular disease. A third of SLE patients can have ocular complications which are important since they can be sight-threatening and may be the presenting features of the disease. The course of the disease is variable as patients have remissions between exacerbations. Women of child-bearing age are commonly affected. Afro-caribbeans and Asians are more likely to be affected.
Ocular Pathophysiology: There is an immune complex deposition in blood vessels of the conjunctiva, retina, choroid, sclera, cornea, ciliary body and in the peripheral nerves of the conjunctiva and ciliary body. Antibody related mechanism can cause vasculitis and thrombosis. Antibody dependent cytotoxicity can cause retinal cell death and demylination of the optic nerve. There are pathogenic antibodies in the circulation which include anti-phospholipid antibodies (APA) and antineuronal antibodies (ANA).
- Subcutaneous nodules
- Rash –There may be a discoid lupus - type rash over the eyelids
- Madarosis – loss of eyelashes. This is quite rare.
- Keratoconjunctivitis sicca – ‘dry eye syndrome’ where there is reduced tear production from the lacrimal glands. This is often associated with secondary Sjögren's syndrome. Irritation and redness may occur. There can also be severe pain and visual loss.
- Peripheral ulcerative keratitis – rare, marker of active systemic vasculitis.
- Episcleritis – superficial inflammation of the sclera, presents with mild redness and irritation as a result of injection of the superficial blood vessels.
- Scleritis – deep inflammation of the sclera, more painful than episcleritis, sight threatening. Anterior scleritis consists of redness due to injection of the deeper episcleral vessels. The redness may be hidden under the upper eyelid so this should be lifted u to check. Anterior scleritis is mostly unilateral and without any discharge. It may be diffuse or nodular in distribution. Posterior scleritis does not cause redness in the eye but can complicate vision, causing blurring, double vision and refraction changes.
Retinopathy may be mild and asymptomatic but if this is severe it can cause visual loss, field defects, floaters and distortion of vision.
- Cotton-wool spots – damage to nerve fibres as a result of swelling on the surface layer of the retina due to infarction. These may be isolated or associated with haemorrhage.
- Intraretinal haemorrhages
- Macular ischaemia
- Roth spots - (white-centred haemorrhages)
- Retinal Vasculitis - inflammation of the retinal blood vessels
- Vascular tortuosity
- Central serous chorioretinopathy - serous detachment of the neurosensory retina where there is leakage from the choriocapillaries.
- Perivascular hard exudates
- Arteriolar occlusion – occurs at the severe stage of the disease. Usually affects the small vessels but the larger vessels can also be affected resulting in branch retinal vein occlusion, central retinal vein occlusion, central retinal artery occlusion or a combination of these.
- Retinal neovascularisation – new vessel growth due to retinal ischaemia.
Optic nerve disease occurs in about 1% of patients with SLE.
- Anterior/Posterior optic neuropathy – bilateral and acute loss of vision which is painless. There may be an arcuate field defect, with or without optic disc swelling. This results from occlusion of the small vessels supplying the optic nerve. This can lead to demylination or axonal necrosis, if severe. Unilateral optic neuropathy is usually associated with thrombosis with the presence of APA.
- Optic neuritis - can present with unilateral loss of vision. It can be painful and made worse with eye movements
- Orbital Myositis – inflammation and enlargement of the intraocular muscles. This is quite rare.
- Orbital ischaemia and infarction – This is rare.
- Periorbital oedema –This is rare
- Orbital Masses - This is rare
- Gaze palsies
- Internuclear opthamoplegia - an impairment of conjugate gaze, where the affected eye fails to adduct.
- Papillitis – a specific type of optic neuritis where there is inflammation in the head of the optic nerve.
- One-and –a-half syndrome – a lesion in the pons causing a failure of lateral conjugate gaze to one side, failure of adduction of the eye on that side and nystagmus when abducting the other eye. This sysndrome is rare with SLE and mainly presents with Multiple Sclerosis.
It is important to be aware that drugs which are used in the treatment of SLE can also enhance ocular effects causing cataract and retinopathy, similar to that seen in Diabetes and Hypertension.
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