Being Open to Alternatives Leads to a Giant Cell Arteritis Diagnosis

Feb 21, 2020 1:58 PM


Dr. Harrie practices comprehensive ophthalmology and directs Moran’s Ophthalmic Ultrasound Department.
Dr. Harrie performs an ophthalmic ultrasound exam.

By Roger P. Harrie, MD

A 78-year-old man was referred for an ultrasound of his right eye to evaluate a small choroidal nevus. He also mentioned almost total loss of vision in his left eye over the past several days. His ophthalmologist attributed this to a central retinal artery occlusion secondary to atrial fibrillation.

During the performance of the ultrasound for the fundus lesion, the patient mentioned the recent onset of severe headaches, temporal scalp tenderness, and pain on chewing, which necessitated changing his diet to soft foods and liquids. Because of this history, an examination was performed, which revealed vision OD of 20/50-2 and OS of light perception with a 4+ afferent pupil defect. 

Medical History

His past medical history was significant for hypertension (Rx Losartan and Triamterene hydrochlorothiazide), coronary artery disease, hyperlipidemia (Rx Atorvastin), hypothyroidism (Rx Levothyroxine), and osteoarthritis (Rx acetaminophen). Asymptomatic atrial fibrillation had recently been discovered on a pre-op evaluation for rotator cuff surgery. A few weeks later, he noted hazy vision in his right eye with lightning streaks. A workup included a brain MRI, which showed chronic microvascular changes, a carotid ultrasound and echocardiogram, both with normal results. He had been started on Eliquis for presumed emboli due to his atrial fibrillation, with resolution of his visual symptoms.

Procedure Possibilities for Giant Cell Arteritis Diagnosis

During the performance of the ultrasound for the fundus lesion, the patient mentioned the recent onset of severe headaches, temporal scalp tenderness, and pain on chewing, which necessitated changing his diet to soft foods and liquids. Because of this history, an examination was performed, which revealed vision OD of 20/50-2 and OS of light perception with a 4+ afferent pupil defect.

Figure 1. Orbital color Doppler with normal orbital blood flow (left) and minimal flow as a result of GCA.
Figure 1. Orbital color Doppler with normal orbital blood flow (left) and minimal flow as a result of GCA.

Intraocular pressures were normal, and the anterior segment examination was unremarkable except for bilateral pseudophakia. Fundus exam was normal OD, but OS showed optic disc pallor and vascular attenuation.

We performed an orbital color Doppler study, which showed a "dead" orbit OS consistent with giant cell arteritis (GCA) (Figure 1). We ordered an erythrocyte sedimentation rate (ESR), which was borderline elevated at 35mm, and a c-reactive protein (CRP), which was highly elevated at 81. We scheduled the patient for a temporal artery biopsy, and he accepted an invitation to participate in a study at the Moran Eye Center using color Doppler to evaluate the temporal artery (Figure 2).

Color Doppler of normal temporal arteries: transverse (left) and cross-section scans.
Color Doppler of normal temporal arteries: transverse (left) and cross-section scans.
Color Doppler of the patient’s temporal arteries. Arrows point to the “halo” sign. Top row: transverse and cross-section scans of the right temporal artery; bottom, transverse and cross-section scans of the left temporal artery.
Color Doppler of the patient’s temporal arteries. Arrows point to the “halo” sign. Top row: transverse and cross-section scans of the right temporal artery; bottom, transverse and cross-section scans of the left temporal artery.

This demonstrated a positive “halo” sign, which was suggestive of inflammatory edema of the temporal arteries.

We referred the patient to the emergency room in his hometown, where he received one gram of intravenous methylprednisolone for three days. A temporal artery biopsy was performed two days later and was positive for GCA. He was started on 60 mg of oral Prednisone per day, with a tapering dosage to be monitored by his primary care physician. He felt his right eye improved slightly, but there was no change in his left eye.

This case demonstrates the importance of the clinician remaining open to alternative diagnostic possibilities when evaluating a patient.

About the Author

Dr. Harrie practices comprehensive ophthalmology and directs Moran’s Ophthalmic Ultrasound Department. He has been the senior instructor in the ocular ultrasound course at the American Academy of Ophthalmology annual meetings and has published numerous articles, book chapters, and two textbooks.

Clinical Trial: Color Duplex Sonography in Evaluating Giant Cell Arteritis

Temporal artery biopsy is the gold standard in the diagnosis of giant cell arteritis (GCA), but color Doppler is being studied as a non-invasive alternative in a new clinical trial at the Moran Eye Center.

While temporal artery biopsy has a high specificity in diagnosis, it lacks sensitivity and carries potential complications that come with undergoing a surgical procedure. Scheduling the procedure can also delay or end the treatment with high-dose corticosteroids, which have frequent and impactful side effects.

Recently, there have been efforts to find a more accessible, less invasive, less costly, and more rapid diagnostic tool. Color duplex sonography (CDS) of the temporal arteries has emerged as such a diagnostic method.

Temporal artery biopsy in cases of GCA reveals inflammation of the vessel wall in a predictable pattern. Similarly, CDS can provide a visual representation of vessel wall edema, referred to as a “halo,” throughout the length of the vessel. CDS also can eliminate the concern for negative temporal artery biopsy results due to “skip lesions.”

Nevertheless, evidence and large population studies are still inadequate to propel CDS as a mainstay of evaluation and diagnosis in GCA. More patients are needed to support further and justify this method of evaluation. With more evidence, CDS could become an integral part of diagnosis in GCA.

-Michael Burrow, MD

Moran is seeking about 100 patients for its study, “Use of Color Duplex Sonography in the Evaluation of Giant Cell Arteritis.” Email michael.burrow@hsc.utah.edu for more information.

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