Statistics from Altmetric.com
Patients with scleroderma experience ophthalmic symptoms related to dry eyes. Involvement of the posterior segment is often subclinical and visual loss as a direct result of the disease is rare. We report for the first time a patient with a bilateral ischaemic retinopathy with neovascularisation that responded to panretinal scatter photocoagulation.
A 51 year old woman with known scleroderma presented with a 9 month history of increasing visual loss. She had presented at the age of 49 with a 4 year history of typical Raynaud’s phenomenon and a 6 month history of symmetrical skin induration affecting her hands, feet, and face.
Subsequently skin sclerosis extended over the chest wall and proximal limbs, typical of diffuse systemic sclerosis. She also had oesophageal involvement and interstitial lung fibrosis. Initially she responded well to symptomatic treatment, but 2 years later she developed worsening breathlessness and pulmonary hypertension was confirmed by right heart catheter.
At the time of presentation to the eye clinic her blood pressure was well controlled and her blood sugars were normal. On examination, best corrected visual acuity was 6/9+2, N6 in the right eye and 6/12+2, N8 left eye. Anterior segment examination and intraocular pressures were normal.
Dilated fundal examination showed bilateral disc neovascularistion, multiple cotton wool spots, and marked venous tortuosity (fig 1). Fluorescein angiography showed marked bilateral capillary closure, disc neovascularisation, and left macular ischaemia (fig 2).
A left panretinal photocoagulation was performed the same day and the right treated similarly 2 weeks later. Two weeks later, there was no objective change in her acuities but fundal examination showed partial regression of the disc new vessels.
She subsequently deteriorated systemically and died of cardiac failure secondary to pulmonary hypertension 30 months after her initial presentation.
Scleroderma targets many organs, including the skin, blood vessels, synovium, gastrointestinal tract, kidneys, heart, and lungs. The lesions of scleroderma are typified by inflammation and microvasculopathy, which in turn stimulate collagen overproduction and fibrosis.
Keratoconjunctivitis sicca is the most common ocular complication of scleroderma, present in up to 70% of cases,1 and may be complicated by foreshortening of the conjunctival fornices.2 Choroidal disease is also common in scleroderma; one series reported that 53% of cases had patchy areas of non-perfusion on fluorescein angiography (FFA), undetectable in all but one, on funduscopy.3 Another study found choriocapillaris abnormalities and normal fundi in seven of 21 FFAs.4
Retinopathy in scleroderma has been described in two patients. The first of these was thought to be related to uncontrolled systemic hypertension, with funduscopic findings of cotton wool spots, oedema, and haemorrhages. The second case, with normal blood pressure and normal fundi, was found to have histopathological changes throughout the retina consisting of extensive vacuolation in the nerve fibre, ganglion cell, and plexiform layers.5
Horan reported the ophthalmological findings in a series of 23 patients; only one patient was found to have a small superficial retinal haemorrhage, in the absence of vascular risk factors.6
Hypertensive retinopathy and branch vein and artery occlusions have been described and it has been noted by several authors that the retinopathy is more florid than would be expected with the level of recorded blood pressure. Our patient developed pulmonary hypertension secondary to a fibroproliferative pulmonary vasculopathy. It is well recognised that other vascular beds (renal, digital, and gut) also have intimal proliferation and fibrosis in scleroderma, and it is likely that this was the pathological process underlying the patient’s eye disease.
To our knowledge, this is the first report of frank bilateral ischaemic retinopathy in association with scleroderma and the first to document neovascularisation at the disc with a beneficial response to laser photocoagulation.