Abstract

Background/Aims

Central retinal artery occlusion (CRAO) is one of the ocular manifestations of giant cell arteritis (GCA), presenting in up to 14% of GCA patients with eye symptoms. Typically, this presentation is unilateral; however, bilateral CRAO is exceptionally rare in GCA. Diagnosis poses challenges, particularly in the subset of GCA patients, termed as occult GCA, who primarily manifest with eye symptoms and may exhibit normal inflammatory markers. A high suspicion of GCA is crucial in cases of bilateral CRAO to avert permanent visual loss (PVL). We present a case of bilateral CRAO with normal inflammatory markers, later confirmed GCA.

Methods

Case report

Results

A 74-year-old lady presented to the eye casualty with visual loss in both eyes upon waking up. Ophthalmological examination revealed bilateral macular ischemia due to bilateral CRAO (Fig). She was referred to medical team for further work-up. She had similar symptoms a couple of weeks prior when vision resolved itself within 24 hours. She also described mild jaw pain on chewing for the last 5 days. Her CRP was 5 (n ≤ 5) and ESR was 21 (n ≤ 30). She was started on prednisolone 60 mg daily with suspicion of GCA. However, further investigations were carried out to find underlying cause. Echocardiogram, ANA, ANCA, and CT head and body were normal. Ultrasound for temporal arteries showed equivocal wall thickening; however, temporal artery biopsy confirmed the diagnosis of GCA. Unfortunately, she could not regain her vision after starting steroids.

graphic

Conclusion

This case highlights the occult presentation of GCA, secondary to the unique features of bilateral CRAO with normal inflammatory markers. Mansfield-Smith et al. (2023) presented 10 years of data from Norwich, showing that 12.5% of GCA patients with visual loss did not exhibit any cranial or systemic features. Only a few cases of bilateral CRAO secondary to GCA have been reported in the literature. Hayreh et al. (1998), in a prospective study, described that patients with occult GCA tend to have significantly lower inflammatory markers compared to those with systemic features. Clinicians, particularly rheumatologists, should remain aware of this rare GCA presentation to facilitate prompt diagnosis and treatment, potentially preventing PVL.

Disclosure

M. Ghaffar: None. S. Thapa: None. H. Abdullah: None. U. Arain: None. D. Makkuni: None.

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