This site is intended for healthcare professionals as a useful source of information on the diagnosis, treatment and support of patients with lupus and related connective tissue diseases.

Introduction

Ocular manifestations of Systemic Lupus Erythematosus (lupus) are unusual. Broadly, they can be divided into those affecting either the front or the back of the eye or as drug effects, particularly those of hydroxychloroquine.

A. Front of the eye

(a) Keratoconjunctivitis sicca

Approximately 25% of lupus patients will have evidence of keratoconjunctivitis sicca (dry eyes). The main symptoms are of gritty, irritable, uncomfortable eyes that may be associated with some redness.Vision is unaffected; there is no pain, photophobia or discharge.

Treatment

Artificial tear substitutes, instilled as drops or a gel, usually give relief of symptoms. When selecting a treatment the main factor to consider is its viscosity. Low viscosity drops require frequent administration (sometimes more than hourly) but have minimal effect on vision. More viscous gels transiently blur the vision but are longer lasting and so may be effective when used only 4-6x/d. Highly viscous paraffin based ointments significantly blur vision and may only be suitable for night time use.A combination of a gel during the day and a paraffin at night is one popular and effective combination (Table 1).

Patients with more severe keratoconjunctivitis sicca who do not respond to these treatments will need to be referred to an ophthalmologist who will consider using preservative-free preparations, physiological tear substitutes (e.g. the hyaluronic acid based preparations), or even plugging the openings of the tear ducts.

(b) Scleritis

This is an unusual but potentially sight threatening condition and may be an important indication of the severity of the lupus. One or both eyes may be involved and patients complain of pain, often so severe that it wakes them at night. There is an area or areas of intense redness with normal visual acuity, no photophobia or discharge.

REFER URGENTLY TO AN OPHTHALMOLOGIST.

Treatment

Mild cases usually require the use of oral non-steroidals. Failure of treatment or severe cases will need systemic immunosuppression in the form of oral corticosteroids often with an immunosuppressant and, in resistant cases, pulsed intravenous methylprednisolone and cyclophosphamide.

(c) Other manifestations

Conjunctivitis and episcleritis have been described but are rare.

Treatment

Conjunctivitis can be treated with a course of antibiotic drops. Episcleritis, which may present with patches of redness, normal vision, no pain or photophobia but occasional discomfort and no discharge, may be a self-limiting condition. Oral non-steroidals, such as flurbiprofen can be effective but should only be prescribed if there are no contra-indications as a result of the lupus. Topical corticosteroids have a role in resistant cases but should normally only be prescribed under ophthalmic supervision.

B. Back of the eye

Despite the well-recognised and documented features described below, these manifestations are unusual.

(a) Retinal Disease

Retinal findings in lupus may result from several pathophysiological mechanisms, including small vessel vasculitis, large vessel occlusive disease, secondary systemic hypertension, and anaemia. Ocular complications tend to occur in acutely ill patients with active system disease.

i. Lupus retinopathy

Classic findings are cotton wool spots and retinal haemorrhages which may be found in 5 to 15% of patients. This microangiopathy probably results from the vasculitis associated with immune complex deposition in the small vessels. A prospective clinical study revealed that 88% of patients with lupus retinopathy had active systemic disease. Furthermore, lupus patients with retinopathy had a significantly decreased survival compared with lupus patients without retinopathy.Visual loss is uncommon and the patients may be asymptomatic.

Treatment

The retinopathy improves with treatment of the systemic disease.

ii. Retinal vasculitis

A few patients with lupus retinopathy develop a severe retinal vasculitis with possible progression to proliferative retinopathy. The visual prognosis is much worse with more than 50% of affected eyes seeing 6/60 or worse.The underlying process is characterised by diffuse arteriolar occlusion with extensive capillary non-perfusion and retinal neovascularisation may result. This may present as a gradual loss of vision, or sudden loss resulting from a vitreous haemorrhage secondary to the retinal neovascularisation. Tractional retinal detachment may occur. Severe retinopathy is typically associated with active systemic disease and with CNS lupus in particular.
Treatment

Immunosuppression, primarily with corticosteroids, is the mainstay of therapy. Laser photocoagulation for proliferative retinopathy (similar to its use in diabetic retinopathy) is felt to be beneficial. Rarely, surgical intervention is required if the vitreous haemorrhage fails to clear or for retinal detachment.

iii. Large vessel occlusive disease

Branch and central retinal vein or arterial occlusions can occur. Arterial occlusions will result in a more profound, usually permanent, visual loss. Arteriolar, particularly branch, occlusions may form part of the anti-phospholipid antibody syndrome. Although venous occlusions often result in permanent loss, some may recover vision with time. Retinal ischaemia may be a complication and retinal neovascularisation may result, particularly after central retinal vein occlusion. Symptoms are of sudden, painless loss of vision.

Treatment

The patient should be monitored for any further complications, such as retinal ischaemia and neovascularization, which would require treatment. In addition, the systemic disease must be adequately controlled.

iv. Hypertensive retinopathy

Typical retinal vascular changes can be seen in those patients who are hypertensive but these changes can be mistaken for lupus retinopathy and vice versa. Patients may be asymptomatic but would complain of sudden central visual loss if they developed a complication, such as a branch retinal vein occlusion.

Treatment

The changes often resolve with reduction of the blood pressure.

v. Anaemia

In some cases, peripheral, blotchy intraretinal haemorrhages may reflect anaemia combined with thrombocytopaenia, rather than the vasculitic component of the disease.

Treatment

The changes often resolve with resolution of the anaemia.

(b) Choroidal Disease

Lupus choroidopathy

Occasionally, the choroid (the layer beneath the retina) can be involved. Lupus choroidopathy results in multifocal serous detachments of the retina and underlying retinal pigment epithelium. These types of non-rhegmatogenous detachments (i.e. with no retinal hole) may be difficult to see with a direct ophthalmoscope. Visual loss is variable depending on the extent of macular involvement.

Treatment

The detachments may regress with improved control of the systemic disease.

(c) Neuro-ophthalmological Disease

Neurological complications of lupus are seen in 25 to 75% of patients. Several neuro-ophthalmological manifestations of lupus have been reported, including ischaemic optic neuropathy and retrobulbar neuritis. Ischaemic optic neuropathy presents with sudden visual loss, often associated with an inferior altitudinal field loss. This type of field loss affecting the horizontal meridian easily distinguishes it from more posterior visual pathway field defects which obey the vertical meridian. The optic disc is pale and swollen.A swollen optic disc in lupus may also be secondary to hypertension, central retinal vein occlusion and increased intracranial pressure from intracranial disease.

Retrobulbar neuritis results in a central or paracentral scotoma, red desaturation, pain on ocular movement and a relative afferent pupillary defect. The optic disc appears normal and the condition may be difficult to differentiate from that seen in association with demyelination. There is an association between neuroophthalmological disease and the anti-phospholipid antibody syndrome. Retrochiasmal visual problems, such as transient amaurosis, visual hallucinations and homonymous field defects have all been described.

Treatment

Systemic corticosteroids are the treatment of choice but although a return of vision would be expected with retrobulbar neuritis, it may not occur after ischaemic optic neuropathy.

ALL BACK OF THE EYE PROBLEMS SHOULD BE REFERRED TO AN OPHTHALMOLOGIST