Graham Hughes1 and Shirish Sangle2
(1)
The London Lupus Centre, London Bridge Hospital, London, UK
(2)
Louise Coote Lupus Unit, St Thomas’ Hospital, London, UK
Abstract
The eye is frequently affected in Hughes Syndrome. The commonest manifestations relate to ischaemic damage in the posterior segment, with retinal and choroidal lesions resulting from either arterial or venous thrombosis, or both.
The eye is frequently affected in Hughes Syndrome. The commonest manifestations relate to ischaemic damage in the posterior segment, with retinal and choroidal lesions resulting from either arterial or venous thrombosis, or both.
The commonest symptom is of transient visual disturbance (transient blurred vision or amaurosis fugax) with diplopia and transient field loss a frequent complaint.
Clinical findings include venous engorgement, occluded central vein or artery and secondary haemorrhage. The ocular symptoms may also result from cerebral infarction in the occipital cortex, patients being left with, for example, permanent bitemporal hemianopia.
13.1 Optic Neuritis
A number of cases of optic neuritis have been described in patients with Hughes Syndrome, as have been cases of Devic’s disease (neuromyelitis optica). This highlights the difficulties in differential diagnosis of Hughes Syndrome from multiple sclerosis (Fig. 13.1).
Figure 13.1
A fluorescent angiography showing retinal hemorrhage and optic disc atrophy in a patient with both Hughes Syndrome and Devic’s syndrome
13.2 Sjögren’s Syndrome
As in many other autoimmune diseases such as Hashimoto’s thyroiditis, there may be associated Sjögren’s syndrome, with a dry Schirmer’s test (impaired tear secretion) and a tendency to conjunctival irritability and infection (Fig. 13.2).
Figure 13.2
Patients with Sjögren’s syndrome often have Sicca syndrome (dry eyes, mouth and vagina). This can be easily tested in eyes by Schirmer’s test (see chap. 23)