Sir,

Giant cell arteritis (GCA) is a systemic inflammatory vasculitis of unknown aetiology, affecting medium and large calibre arteries. It can result in profound visual disability that usually occurs from occlusion of the posterior ciliary arteries leading to anterior ischaemic optic neuropathy (AION). Visual loss can also result from occlusion of any other arteries supplying the visual pathway. We report a case of gross, chronic, posterior segment ocular ischaemia with excellent visual recovery following treatment with systemic steroids.

Case report

An 83-year-old man presented with a 5-month history of decreased visual acuity (VA) in the right eye.

On examination, Snellen acuities were 6/60 (OD) and 6/6- (OS). There was no anterior segment neovascularisation. The right fundus was pale with multiple cotton wool spots. There were a small number of cotton wool spots on the left fundus. Significant venous tortuosity was absent and there were no haemorrhages in either side.

Fluorescein angiography (FFA) was performed and demonstrated grossly delayed choroidal filling and areas of absent choroidal filling even in the late phase. There were also dilated retinal veins without tortuosity and delayed venous filling (Figure 1 top). Biochemical investigation revealed raised inflammatory markers (c-reactive protein (CRP) of 137 mg/l and plasma viscosity of 2.02 mPA s). On further enquiry, the patient admitted a 6-month history of myalgia, temporal headache, jaw claudication, and weight loss. It was noted that a diagnosis of chronic confusion, sepsis, and cerebral ischaemia had been made by the care of the elderly physicians following a collapse 5 months earlier, and that the CRP was elevated to 103 when the patient was admitted on that occasion. These findings were attributed to a lower respiratory tract infection. On discharge from elderly care, the diagnosis was ‘collapse and chronic confusion’; CRP was 74.

Figure 1
figure 1

Top: Pretreatment FFA. In clockwise order from top right 32, 36, and 53 s. Bottom: post-treatment FFA. In clockwise order from top right 19, 25, and 34 s.

Full size image

A diagnosis of chronic GCA (later biopsy proven) and choroidal ischaemia was made by us and the patient was admitted and treated with intravenous metyhlprednisolone and oral prednisolone. Temporal artery biopsy revealed granulomatous cell infiltration, proliferation of the intima, and disruption of the internal elastic lamina typical of GCA.

Vision improved to 6/9 (OD), 6/6 (OS) within 2 days and inflammatory markers normalised. The patient was discharged to outpatients on oral prednisolone.

A post treatment FFA (Figure 1 bottom) was performed and revealed dramatic improvement in perfusion at all levels.

Comment

Choroidal ischaemia is an uncommon consequence of GCA,1 with an incidence of one in 170 in one case series. 2 The posterior ciliary arteries are most commonly affected. It is notable that in this case AION did not occur despite severe choroidal ischaemia. Choroidal ischaemia is much more common in cases of carotid artery insufficiency due to atherosclerotic disease. Despite carotid endarterectomy and resumption of blood flow in these cases, significant visual recovery is rare.3

This case demonstrates that, contrary to popular opinion, GCA-induced ischaemia can be successfully reversed with treatment. As long as irreversible end-organ damage has not occurred (choroidal ischaemia vs choroidal infarction, or more commonly AION), treatment can restore perfusion and end-organ function.