Ophthalmic Manifestations of Giant Cell Arteritis

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Ophthalmic Manifestations of Giant Cell Arteritis

  1. Giant Cell Arteritis

    1. Giant Cell Arteritis (GCA) is an Immune-Mediated Vasculitis

    2. Affects Medium and Large Arteries

      1. In eye mostly Posterior Ciliary Artery (PCA), then Central Retinal Artery (CRA), rarely Ophthalmic Artery (OA). Never Branch Retinal Artery.

      2. Explain why GCA affects eyes

      3. Called GCA because of granulomatous inflammation. Arteries lined with multinucleated giant cells: macrophages, lymphocytes, and fibroblasts

    3. Incidence of 2.3/100,000 in 6th decade. Increases with Age.

    4. Happens mostly to those > 50 years old

      1. The higher the age, higher incidence

    5. Predilection for North European, Scandinavian descent. Typically Caucasian though can happen in other races

    6. Why is it important?

      1. Profound vision loss

        1. Causes A-AION

          1. Can become bilateral

        2. Treatable

  1. Arteritic Anterior Ischemic Optic Neuropathy (A-AION)

    1. A-AION

      1. Sudden painless vision loss

      2. Unilateral Optic Disc Edema

      3. 1/10 patients (> 50 years old) with optic disc edema are A-AION. Other 9/10 NA-AION

      4. Can become bilateral in 14 days in 1/3 if untreated

    2. Systemic Symptoms

      1. Jaw Claudication (Odds Ratio 9.0)

      2. Neck Pain (Odds Ratio 3.4)

      3. Anorexia

      4. Less predictable symptoms

        1. Headache

        2. Fever

        3. Scalp Tenderness

        4. Malaise

      5. Average number of symptoms = 3

Giant cell arteritis (GCA) is an immune-mediated vasculitis with serious ophthalmic manifestations. The most common is Arteritic Ischemic Optic Neuropathy (AION). Patients can also suffer from other visually disabling conditions such as Central Retinal Artery Occlusion (CRAO) and diplopia. It is important for the clinician to understand the signs and symptoms of GCA and how they relate to the eye in order to diagnose and prevent visual loss.


Understand signs and symptoms of Giant Cell Arteritis

Understand laboratory evaluation for Giant Cell Arteritis

Discuss diagnostic strategies for diagnosing Anterior Ischemic Optic Neuropathy

Discuss diagnostic strategies for diagnosing Central Retinal Artery Occlusion with GCA

Discuss diagnostic strategies for diagnosis diplopia from GCA

    1. Visual Symptoms/Signs

      1. Sudden, painless vision loss

      2. 31% had amaurosis fugax 1-2 weeks before

        1. This can be misleading. Not saying 31% of patients with Amaurosis Fugax have GCA

      3. 6% diplopia

      4. 8% ocular pain

      5. Optic Disc Edema “chalky white pallor”

    2. Lab Testing

      1. ESR – 86% sensitive

      2. CRP – 100% sensitive, 82% specific

      3. Combined ESR and CRP – 99% sensitive, 97% specific

      4. CBC with differential looking for disease that can affect Red Blood Cells (i.e. Anemia, Polycythemia Vera)

    3. Other Diagnostic Testing

      1. Fluorescein Angiography (FA) – characteristic late filling of choroid in the 2 weeks after Optic Disc Edema starts

      2. Ultrasound, PET, MRI of limited benefit

      3. Temporal Artery Biopsy – especially indicated if clinical symptoms highly suggestive but either ESR or CRP are inconclusive

    4. This is a diagnosis that can be made by Optometrist or Ophthalmologist with use of ocular signs and symptoms combined with systemic symptoms and lab testing. Additional testing often not needed.

    5. Contralateral optic nerve can give us clues to A-AION vs. NA-AION

      1. Go through Bayesian analysis

        1. If C/D is > 0.4 1/5 have A-AION

        2. If C/D is < 0.3 1/15 have A-AION

    6. Some advocate American College of Rheumatology criteria

      1. Need 3 of the following 5 criteria

        1. Over 50 years of age

        2. New onset of Headache

        3. Scalp tenderness or decreased temporal artery pulse

        4. ESR > 50 mm/h

        5. (+) Temporal Artery Biopsy

      2. Having 3 of these 5 gives 94% sensitivity and 91% specificity (not good enough)

    7. Treatment

      1. Most studied and likely still most effective is oral steroid

        1. 80-100 mg/day until labs normalize

          1. Does not typically improve vision (only 4% improve)

        2. Then taper for VERY long (years sometimes!)

        3. Evidence still lacks because not ethical to have a placebo group

      2. No evidence that IV steroid in mega dose any more effective

      3. Limited evidence for TNF blockers, Methotrexate and other immune modulators

    8. Clinical Picture

      1. Unilateral Optic Nerve Edema

      2. Systemic Symptoms

      3. Possible Visual Symptoms

      4. Lab Results

        1. ESR > 47 mm/h

        2. CRP > 2.45 mg/dl

      5. Optic Nerve in other eye > 0.4

      6. Positive Temporal Artery Biopsy

  1. Central retinal artery occlusion (CRAO) can occur

      1. CRAO cause by GCA 5-10%

      2. Important to understand this and run labs

      3. Can also get Cilioretinal artery occlusion

        1. This is due to PCA inflammation

        2. Often confused with BRAO

  2. Diplopia

    1. Hayreh found around 6% have diplopia

    2. When patient over 50 has new onset CN palsy, should ask about GCA symptoms

    3. Consider labs

    4. Should always consider GCA if

      1. Correct age

      2. Other neuro symptoms

      3. Atypical presentation

      4. Patient is not diabetic or hypertensive

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