Elderly man presents with bilateral decreased vision
Moderate optic disc edema and optic nerve hemorrhages were greater in the right eye than the left.
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An 83-year-old man presented to the New England Eye Center with a 2-week history of decreased vision in both eyes. He felt like letters were “clumping together,” making it difficult to read. He also complained that the inferior half of his vision in the right eye was foggy. He denied any new headaches, jaw claudication, transient visual obscurations, scalp tenderness, recent weight loss or fatigue. He was experiencing new gait instability and poor coordination.
His medical history included congestive heart failure, paroxysmal atrial fibrillation on coumadin, hypertension, hyperlipidemia, hypothyroidism, benign prostatic hypertrophy and sinoatrial node dysfunction for which he underwent pacemaker placement. He was recently started on amiodarone for his atrial fibrillation. He had a history of hyperopia and astigmatism for which he wore glasses, idiopathic anterior uveitis of the right eye (last episode occurred 1 year before presentation), senile cataracts in both eyes and evaporative dry eye syndrome in both eyes. The patient was a retired physician who did not smoke cigarettes or drink alcohol.
Source: Alison Lauter, MD, and Laurel Vuong, MD
Examination
On examination, the patient’s visual acuity with correction was 20/80 bilaterally. Color vision with Ishihara color plates was diminished in both eyes. Pupils were equal and briskly reactive to light with no afferent pupillary defect. IOP was 14 mm Hg in both eyes. Extraocular movements were full in both eyes.
Humphrey visual field 30-2 SITA Fast testing showed diffuse depression in the right eye and superior and inferior arcuate defects in the left eye (Figure 1). Anterior slit lamp examination was notable for moderate nuclear sclerotic and cortical cataracts with no evidence of anterior chamber cell or keratic precipitates. Dilated fundus exam revealed moderate optic disc edema and optic nerve hemorrhages in the right eye greater than the left (Figure 2). No retinal hemorrhages or lesions were noted. OCT of the macula was normal in both eyes. OCT of the retina nerve fiber layer confirmed bilateral disc edema. Fluorescein angiography in both eyes showed delayed filling time with peripapillary blockage but no optic disc leakage.
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Bilateral decreased vision
Bilateral optic disc edema and optic nerve hemorrhages in an elderly patient are concerning for giant cell arteritis, bilateral sequential non-arteritic anterior ischemic optic neuropathy, optic neuropathy due to amiodarone toxicity, an intracranial process and infectious/inflammatory optic neuropathy. The patient did not experience systemic symptoms concerning for giant cell arteritis, but given his age and clinical presentation, it was essential to rule out giant cell arteritis.
Case continued
Any patient with concern for giant cell arteritis should be evaluated as soon as possible with an erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). The CRP for our patient was within normal limits, and the ESR was mildly elevated but within normal limits when adjusted for age. Additional labs obtained as an outpatient, including Lyme antibody, lysozyme, angiotensin converting enzyme and treponemal antibody, were negative or normal. The patient was tapered off amiodarone by his cardiologist given concern for amiodarone optic neuropathy, although it was unlikely given the recent initiation.
Due to the bilateral optic disc edema and vision loss, the patient was admitted to the hospital to expedite further workup. An MRI of the brain and orbits with and without contrast and FLAIR sagittal showed no orbital or intracranial masses and no optic nerve enhancement. Despite the lack of systemic symptoms and borderline elevated ESR level, the patient’s age and bilateral involvement were highly suspicious for giant cell arteritis given the rest of his negative workup. He was started on daily doses of 1 g IV methylprednisolone once the MRI came back negative, and a temporal artery biopsy was planned for during the admission.
Once starting the steroids, the patient noted subjective improvement in his vision, and his visual acuities improved to 20/50 in both eyes. The temporal artery biopsy pathology showed focal increase of histiocytes in the vessel wall and focal disruption of the internal elastic lamina consistent with giant cell arteritis (Figure 3). He received 3 days of IV methylprednisolone and was discharged on high-dose oral prednisone. He followed up with rheumatology as an outpatient and was subsequently started on tocilizumab, a biologic medication used to treat giant cell arteritis and reduce the duration of steroid treatment. Given his response to steroids and the positive biopsy, amiodarone was restarted by his cardiologist.
Discussion
Giant cell arteritis (GCA) is a systemic inflammatory vasculitis of medium- and large-sized arteries most commonly affecting the superficial temporal artery, ophthalmic artery, posterior ciliary arteries and vertebral arteries. Ischemic complications occur as the vasculitis leads to luminal occlusion. GCA typically occurs in the elderly population with peak incidence between the ages of 70 and 80 years. The majority of patients affected are women, accounting for 65% to 75% of cases. GCA often presents with systemic symptoms such as unilateral or bilateral headaches localized to the temple or occipital region, fatigue, myalgias, fever, weight loss, jaw claudication or scalp tenderness. A variety of systemic, neurologic and ophthalmologic complications can occur due to the disease. Rapidly progressive visual loss is the most severe ocular complication and can become permanent. Partial or complete vision loss in one or both eyes occurs in 20% of patients due to retinal artery occlusions and anterior ischemic optic neuropathy. Ischemic optic neuropathy in GCA causes vision loss in about 10% to 15% of patients. Patients can present with ocular symptoms but no systemic complaints, such as our patient, which is termed occult GCA. Occult GCA can occur in up to 38% of cases. Prompt diagnosis and treatment of GCA are paramount to preserving vision and preventing life-threatening complications.
The initiation of systemic corticosteroids in any patient with high suspicion of GCA is vital for preservation of the patient’s vision. Intravenous high-dose steroids should be started in patients with vision loss or a concern for impending vision loss. Workup should include lab work for inflammatory biomarkers such as ESR and CRP. A temporal artery biopsy (TAB) should be performed within 2 weeks of initiating corticosteroid treatment. Obtaining a unilateral biopsy of at least 2 cm in length has been shown to be sufficient in making a diagnosis and is the preferred practice at our institution. A positive TAB will show a mononuclear cell infiltration, granulomatous inflammation with multinucleated giant cells surrounding the vessel and disruption of the internal elastic lamina. In a subset of patients, the ESR and CRP are not elevated but the patient has a positive TAB, as in our case. About 4% of patients with a positive biopsy for GCA will not have elevation of inflammatory markers. The combination of the two biomarkers provides better specificity and sensitivity and is associated with greater odds of a positive TAB than ESR or CPR alone.
Treatment with systemic corticosteroids involves an integrated approach with rheumatology, which manages the dosing and taper of the oral steroids. The patient should be followed closely by his or her primary care physician for the systemic side effects of prolonged steroid intake, such as elevated blood sugars, hypertension and osteoporosis. More recently, biological agents have been shown to have a corticosteroid-sparing effect in the treatment of GCA. Tocilizumab, an IL-6 inhibitor, was the first biologic approved in 2017 for the treatment of GCA. Tocilizumab is often used in patients who have failed treatment with corticosteroids or cannot tolerate long-term corticosteroid use.
Case continued
Our patient was followed closely by the neuro-ophthalmology and rheumatology services. Oral prednisone was gradually tapered, and monthly tocilizumab injections at 4 mg/kg were continued. His bilateral optic nerve edema gradually resolved. His visual acuity at last follow-up was 20/50 in both eyes with residual visual field loss and mild pallor of bilateral optic nerves. OCT of the retina nerve fiber layer and ganglion cell layered showed thinning in both eyes. Our patient has also been followed by a low vision specialist.
This case highlights the importance of prompt corticosteroid initiation and obtainment of a TAB in patients with high clinical suspicion for GCA despite normal levels of inflammatory biomarkers.
- References:
- Buttgereit F, et al. JAMA. 2016;doi:10.1001/jama.2016.5444.
- Danesh-Meyer HV, et al. J Neuroophthalmol. 2000;doi:10.1097/00041327-200020030-00011.
- Kermani TA, et al. Semin Arthritis Rheum. 2012;doi:10.1016/j.semarthrit.2011.10.005.
- Parik M, et al. Ophthalmology. 2006;doi:10.1016/j.ophtha.2006.05.020.
- Sammel AN, et al. Curr Opin Ophthalmol. 2018;doi:10.1097/ICU.0000000000000528.
- For more information:
- Alison Lauter, MD, and Laurel Vuong, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Alison J. Lauter, MD, and Sarah E. Thornton, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.