A 90 year old woman with painless vision loss
Digital Journal of Ophthalmology 2005
Volume 11, Number 7
February 10, 2005
Printer Friendly

Qasim Mansoor | The Lancashire University teaching Hospital UK
Tahir Majeed | The Lancashire University Teaching Hospital UK

A 90 year old woman presented with sudden, painless loss of vision in her left eye on wakening in the morning 3 days prior. She had no associated symptoms of headache, scalp tenderness, jaw claudication, weight loss, or aches or stiffness of the limbs. Further ophthalmic and systemic inquiry was negative. There was no history of hypertension, diabetes, rheumatoid arthritis, or other autoimmune disease.

On examination, her best-corrected visual acuity was 6/18 in the right eye and perception of light in the left eye. There was marked relevant afferent pupillary defect (RAPD) on the left side. The extraocular movements were normal. Anterior segment of both eyes showed moderate cataracts. The intraocular pressures in both eyes were within the normal range. The left optic disc was swollen on fundoscopy and the right disc was normal appearing with a cup to disc ratio of 0.3. The superficial temporal arteries were pulsatile and non-tender.

Ancillary Testing
Radiographic Studies
ESR on initial presentation was 37mm/hr.
Hemoglobin was 13.5 and platelets were 432.
A diagnosis of anterior ischemic optic neuropathy (AION) was made. Although the clinical features were not typical of giant cell arteritis (GCA), in view of the marked visual loss in the left eye this possibility was reconsidered after 48 hours.
Repeat ESR after 48 hours was 53mm/hr and CRP was 84mg/L.
Marked patchy choroidal ischaemia and leakage at the left optic disc was noted on fundus flourescein angiography (Fig I).

Figure 1

A diagnosis of ischemic optic neuropathy due to silent giant cell arteritis was made. She was admitted in the hospital and was given high dose of steroids (I/V methyl prednisolone 500mg stat and prednisolone 80mg/day)
Temporal artery biopsy was performed and showed infiltration of the tunica adeventitia with chronic inflammtory infiltrate including giant cells (Fig 2), confirming the diagnosis of giant cell arteritis.

Figure 2
Arrow points to giant cell.

Differential Diagnosis
Anterior ischemic optic neuropathy arteritic type.
Anterior ischemic optic neuropathy non-arteritic type.
Optic neuritis.
Optic nerve head infiltration (infectious/non infectious/neoplastic).

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.

1. Font C, Cid MC, Coll-Vincent B et al. Clinical features in patients with permanent visual loss due to biopsy proven giant cell arteritis. Br J Rheumatol 1997;36:251-254.
2. Simons RJ, Cogan DG. Occult temporal arteritis. Arch Ophthalmol 1962; 68:39-45.
3. Hayreh SS, Podhajsky PA, and Zimmerman B. Occult giant cell arteritis" ocular manifestations. AJO 1998;125:521-6.
4. Liu NH, LaBree LD, Feldon SE, et al. The epidemiology of giant cell arteritis: A 12 year retrospective study. Ophthalmology 2001; 108:1145-1149.
5. Gurwood AS and Malloy KA. Giant cell arteritis. Clin Exp Op. 2003;85:19-26.
6. Hayreh SS, Podhajsky PA, Raman R et al. Giant cell arteritis: Validity and reliability of various diagnostic criteria. Am J Ophthalmol 1997;123:285-296.
7. Zweegman S, Makkink, Stehouwer C.D.A. giant –cell arteritis with normal erythrocyte sedimentation rate: Case report and review of the literarture 1993;42: 128-131.
8. Hayreh SS. Acute ischemic disorders of the optic nerve: pathogenesis, clinical manifestations and management. Ophthalmol Clin North Am 1996;9:407-442.
9. Danesh-Meyer HV, Savino PJ, and Sergott RC. The prevalence of cupping in end-stage arteritic and non-arteritic anterior ischemic optic neuropathy. Ophthalmol 2001;108:593-8.
10. Matteson EL et al. Long term survival with giant cell arteritis in the American Collge of Rheumatology Giant Cell Arteritis Classification Criteria Cohort. Am J Med 1996;100:193-6.