Case 1

A 74-year-old Green Bay Packers Cheerleader

Q1

A 74 yo ♀ presents to the ED, where you are the physician on call. She is concerned about sudden vision loss in her left eye.

What additional history do you want to obtain?

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[accordion-item title=”Duration of symptoms? (click to show/hide)”]

Oh, well, it all started about 4 hours ago. Is something terrible happening? Am I going to go blind?

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[accordion-item title=”Any history of previous symptoms?  (click to show/hide)”]

Oh, no! I’ve never had anything like this before. I’m really quite worried!

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[accordion-item title=”Occupation?   (click to show/hide)”]

Well, I’m a retired Green Bay Packers cheerleader. Now, I’ve taken up woodworking

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[accordion-item title=”Associated symptoms?    (click to show/hide)”]

Well, I have been having this headache for the past 2 weeks. It’s kind of annoying.

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[accordion-item title=”Eye pain?  (click to show/hide)”]

No, thankfully I don’t have any eye pain. Just headaches.

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[accordion-item title=”History of cardiovascular disease or cerebrovascular accident?  (click to show/hide)”]

No, I’ve always been very healthy. My doctor did say I had high cholesterol, but once I started that tablet, it got better.

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[accordion-item title=”History of trauma?  (click to show/hide)”]

No, I haven’t had any accidents or falls recently. I try to stay away from dangerous situations since I broke my hip 3 years ago.

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[accordion-item title=”Family history of any eye conditions?  (click to show/hide)”]

Not that I know of. My father had cataracts, but he was older – about my age now, when he got them.

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Q2

A 74 yo ♀ presents to the ED, where you are the physician on call. She is concerned about sudden vision loss in her left eye. She states that the episode occurred about 4 hours ago.

She has had no symptoms in the right eye. There is no eye pain, but she notes that she has been having headaches for the past 2 weeks.

What is your differential diagnosis? Pick your top four choices.

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[accordion-item title=”Acute angle closure glaucoma (click to show/hide)”]

Incorrect: Angle closure glaucoma presents with constant pain +/- vomiting, nausea, and abdominal cramps.

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[accordion-item title=”Central retinal artery occlusion (click to show/hide)”]

Correct: Central retinal artery occlusion is associated with sudden painless loss of vision. Associated risk factors include hypertension, hypercholesterolemia, diabetes, vascular disease, prior MI, transient ischemic attacks, and stroke.

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[accordion-item title=”Transient monocular vision loss (click to show/hide)”]

Correct: As the name suggests, transient monocular vision loss causes sudden painless loss of vision. This may be benign in a person younger than 45 years old.

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[accordion-item title=”Dense cataract (click to show/hide)”]

Incorrect: Cataracts are typically slow growing.

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[accordion-item title=”Giant cell arteritis (click to show/hide)”]

Correct: Giant cell arteritis classically presents in an older person with sudden permanent or transient visual loss. Associated symptoms often include headache, jaw claudication, scalp pain, and polymyalgia rheumatica.

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[accordion-item title=”Herpes simplex keratitis (click to show/hide)”]

Incorrect: Herpes simplex keratitis causes a superficial infection of the cornea, often in a dendritic pattern. Symptoms include red eye, photophobia, and some degree of eye irritation.

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[accordion-item title=”Idiopathic intracranial hypertension (click to show/hide)”]

Incorrect: Pseudotumor cerebri classically occurs in young obese females, and produces bilateral visual changes due to increased intracranial pressure. Visual field is usually affected late in disease. Associated with headache worse on lying down and relieved by standing.

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[accordion-item title=”Central retinal vein occlusion (click to show/hide)”]

Correct: Central retinal vein occlusion can present with sudden painless loss of vision. Risk factors include hypertension, diabetes, cardiovascular disease, and hyperviscosity states such as polycythemia.

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Q3

You examined this patient’s eyes using a direct ophthalmoscope. The right eye was completely normal to examination. The left eye is pictured below. What is the most likely diagnosis?

Match the fundoscopic change to the pathological condition.

 

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[accordion-item title=”Central retinal artery occlusion  (click to show/hide)”]

Incorrect: Central retinal artery occlusion has the classic findings of a pale, swollen optic disk, a pale retina with prominent ‘cherry red’ macula.

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[accordion-item title=”Normal retina (click to show/hide)”]

Incorrect: The optic disk seen in this patient’s eye is pale and edematous, which is consistent with ischemic optic neuropathy.

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[accordion-item title=”Central retinal vein occlusion (click to show/hide)”]

Incorrect: Central retinal vein occlusion has the classic findings of retinal hemorrhages, dilated torturous retinal veins, cotton wool spots and retinal and macular edema. Classically described as a ‘blood and thunder’ or ‘stormy sky’ appearance.

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[accordion-item title=”Ischemic optic neuropathy (as occures in GCA) (click to show/hide)”]

Correct: The optic disk seen in this patient’s eye is pale and edematous, which is consistent with ischemic optic neuropathy.

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[accordion-item title=”Retinal detachment (click to show/hide)”]

Incorrect: The retina in this patient is all in one plane with no signs of detachment.

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Q4

You suspect she has giant cell arteritis based on her associated headaches which were located in the temporal region on both sides.

Which other symptoms would you expect her to have if she did?

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[accordion-item title=”Pain & weakness in arms and shoulders (click to show/hide)”]

Correct: The finding (polymyalgia rheumatica) describes proximal muscle weakness, and occurs in 40-50% of patients with GCA.

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[accordion-item title=”Rash on her cheeks (click to show/hide)”]

Incorrect: Malar rash is not a common finding in GCA. If a malar rash was present, a diagnosis of systemic lupus erythematosus would have to be entertained.

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[accordion-item title=”Loss of hair in patches (click to show/hide)”]

Incorrect: Alopecia is not a common finding in GCA. It is also a fairly non-specific finding.

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[accordion-item title=”Pain with chewing (click to show/hide)”]

Correct: Jaw claudication is present in 50% of patients with GCA. This may be present with weight loss, or the patient complaining about not being able to chew steaks any more.

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[accordion-item title=”Dry eyes and mouth (click to show/hide)”]

Incorrect: Xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes) are not commonly associated with GCA. They are however found together in Sjogren’s syndrome.

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Q5

What diagnostic tests would confirm a diagnosis of giant cell arteritis?

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[accordion-item title=”Anti-nuclear antibody (click to show/hide)”]

Incorrect: ANA is positive in many automimmune conditions, including systemic lupus erythematosus, but not in GCA.

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[accordion-item title=”Temporal artery biopsy (click to show/hide)”]

Correct: Temporal artery biopsy is the gold standard for diagnosis of GCA. Sample needs to be ≥ 2 cm in length.

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[accordion-item title=”C-reactive protein (click to show/hide)”]

Correct: CRP is usually elevated in inflammatory processes, including GCA.

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[accordion-item title=”Complete blood count (click to show/hide)”]

Correct: Platelets can commonly be elevated in cases of giant cell arteritis.

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[accordion-item title=”Eryhrocyte sedimentation rate (click to show/hide)”]

Correct: ESR is usually quite elevated in cases of GCA, often >70 mm/hr.

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[accordion-item title=”Anti-neutrophil cytoplasm antibody (click to show/hide)”]

Incorrect: ANCA is useful for the diagnosis of small and medium vessel vasculitides, but has no role in the diagnosis of GCA.

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Q6

In the ED, her ESR and CRP come back elevated at 100 mm/hr and 12 ng/mL respectively. Temporal artery biopsy is not available for the next two days as your surgeon is on vacation.

What is the next best step in the management of this patient?

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[accordion-item title=”Wait until the temporal artery is biopsied in two days, then start high dose prednisone (40-60 mg/day) (click to show/hide)”]

Incorrect: For suspected GCA, the standard of care is to start high dose steroids (usually prednisone) immediately. Temporal artery biopsy will not be altered by steroid use for up to 7-10 days.

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[accordion-item title=”Wait until the temporal artery is biopsied in two days, then start low dose prednisone (5-10 mg/day) (click to show/hide)”]

Incorrect: For suspected GCA, the standard of care is to start high dose steroids (usually prednisone) immediately. Temporal artery biopsy will not be altered by steroid use for up to 7-10 days.

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[accordion-item title=”Start broad spectrum antibiotics – ciprofloxacin, piperacillin/tazobactam, and vancomycin now, wait until the temporal artery is biopsied, then start high dose prednisone (40-60 mg/day) (click to show/hide)”]

Incorrect: There is no role for antibiotics in the treatment of GCA as there is no infection.

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[accordion-item title=”Start broad spectrum antibiotics – ciprofloxacin, piperacillin/tazobactam, and vancomycin now, wait until the temporal artery is biopsied, then start low dose prednisone (5-10 mg/day) (click to show/hide)”]

Incorrect: There is no role for antibiotics in the treatment of GCA as there is no infection.

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[accordion-item title=”Start high dose prednisone now (40-60 mg/day) (click to show/hide)”]

Correct: For suspected GCA, the standard of care is to start high dose steroids (usually prednisone) immediately. Temporal artery biopsy will not be altered by steroid use for up to 7-10 days.

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[accordion-item title=”Start low dose prednisone now (5-10 mg/day) (click to show/hide)”]

Incorrect: For treatment of GCA, high dose prednisone (40-60 mg/day) is the treatment of choice.

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Summary

Giant cell arteritis (GCA) is a granulomatous vasculitis of medium sized blood vessels. It occurs most commonly in females than males with a ratio of about 3:1, and occurs most commonly at around age 70.

Symptoms include headache and scalp tenderness, usually in the temporal region, jaw claudication, amaurosis fugax (transient monocular loss of vision in one eye), and polymyalgia rheumatica (weakness in proximal muscle groups of upper and lower limbs).

The gold standard for diagnosis is temporal artery biopsy (need at least 3 cm sample). Histology reveals non-caseating granulomas in the vessel walls. Lesions are not continuous, so a negative biopsy does not guarantee that disease is not present. Other lab tests include markedly elevated ESR and CRP, along with mildly elevated alkaline phosphatase and platelets.

Treatment should be started immediately as the possible sequelae of untreated GCA include irreversible monocular blindness secondary to ischemic optic neuropathy. High dose prednisone (40-60 mg prednisone daily) should be started immediately, and the results of the temporal artery biopsy will not be affected for the first three days of therapy.