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A 90 year old woman with painless vision loss
Digital Journal of Ophthalmology 2005
Volume 11, Number 7
February 10, 2005
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Qasim Mansoor | The Lancashire University teaching Hospital UK
Tahir Majeed | The Lancashire University Teaching Hospital UK
Diagnosis and Discussion
Occult giant cell arteritis may manifest through ocular symptoms and signs alone, preceding the systemic manifestations often by days and weeks or without systemic features (1-3). Giant cell arteritis, also known as temporal arteritis or cranial arteritis is an inflammatory disease of large and medium sized arteries, often involves the ciliary arteries resulting in an ischaemic optic neuropathy. The severity and extent of involvement are associated with the quantity of elastic tissue in the vessel wall. Giant cell arteritis is more common in Caucasians than other races with an annual incidence range 0.49-23.3/100,000 population. (4) Gurwood et al documented GCA in the setting of normal ESR in up to 9% of cases. (5) Hayreh et al studied 85 patients over a period of twenty-two years and found that 21.2% of patients suffered from occult form of giant cell arteritis without systemic features. (3) They also suggested diagnostic criteria to differentiate arteritic (occult GCA) from non-arteritic anterior ischaemic optic neuropathy:
i. Transient visual obscurations and/or early marked visual loss;
ii. Elevated ESR and C-reactive protein;
iii. Chalky white disc oedema and/or cilioretinal and posterior ciliary artery occlusion on flourescein angiogram; and
iv. Histological findings of arteritis.
None of the above criteria has 100% reliability. Normal ESR does not exclude giant cell arteritis. C-reactive protein is more reliable than ESR. The combination of both gives the best specificity (97%) for the diagnosis of this condition. (3, 6-8)
The most common ocular complication of GCA is ischemic optic neuropathy secondary to ischemia of the ciliary arteries. Other complications may include central retinal artery occlusion and posterior ischemic optic neuropathy. In the initial stages when the disc is edematous, the AION nerve appears chaly-white; within 6-8 weeks, atrophy develops and most patients show a pale and cupped disc. (8,9).
In the absence of systemic features of giant cell arteritis it becomes difficult to make a diagnosis of anterior ischaemic optic neuropathy of arteritic type, as was the case in our patient. She woke up with marked painless visual loss without any systemic feature of this condition. Initial ESR was also normal for her age. Repeat ESR and raised CRP raised the suspicion of GCA and it was confirmed subsequently on histology. When treated with systemic steroids the life expectancy for patients with GCA is virtually the same as for the general population. This illustrates the extreme importance of early diagnosis and treatment of GCA. (10)
Therefore, we emphasize that C-reactive protein should be requested along with ESR to enhance the specificity of the diagnosis in any patient aged more than fifty years presenting with sudden visual loss. The fundus flourescein angiogram and temporal artery biopsy should be considered in suspected cases of giant cell arteritis.