Ophthalmology
Question 52 of 180
A 76 year old woman presents to the Emergency Department with a 2 day history of left sided headache. She describes pain in the temporal region that is exacerbated by touch. In the past 12 hours she has noticed her vision is worsening in her left eye. How is the most likely diagnosis definitively diagnosed?
Answer:
Temporal artery biopsy is the definitive test, which all patients with suspected GCA should undergo. The biopsy is performed on the side with abnormal clinical findings (if present). However, if the biopsy on one side is normal, a second biopsy on the contralateral side should be performed. An adequate length of temporal artery (3 to 5 cm) should be obtained because inflammatory lesions may be present in a segmental fashion. Treatment should not be delayed while waiting for the biopsy to be performed. After several weeks of glucocorticoid therapy, temporal artery biopsy may still yield a diagnosis of arteritis.Giant Cell Arteritis
Neurology / Ophthalmology
Last Updated: 4th July 2021
Giant cell arteritis (or temporal arteritis) is a type of chronic vasculitis characterised by granulomatous inflammation in the walls of medium and large arteries. The extracranial branches of the carotid artery and branches of the ophthalmic artery, such as short ciliary branches, are preferentially involved, although the aorta and its major branches may also be affected.
Clinical features
- Suspect giant cell arteritis if the person is aged 50 years or older with at least one of:
- A new onset localised headache that is usually unilateral, in the temporal area, but is occasionally diffuse or bilateral.
- A temporal artery abnormality, such as tenderness, thickening, or nodularity (45–75% of cases)
- Other symptoms and signs suggestive of giant cell arteritis include:
- Systemic features: low grade fever, fatigue, anorexia, weight loss, and depression (occurs in most cases)
- Features of polymyalgia rheumatica: bilateral upper arm stiffness, aching, and tenderness; and pelvic girdle pain (40% of cases)
- Scalp tenderness, especially over the temporal and occipital arteries (50% of cases)
- Intermittent jaw claudication, causing pain in the jaw muscles while eating (nearly 50% of cases)
- Visual disturbances:
- Permanent partial or complete loss of vision in one or both eyes occurs in up to 20% of cases and is a common early symptom.
- Typically it is described as a feeling of a shade covering one eye, which can progress to total blindness. The eye is not painful.
- Double vision, visual field defects, changes to colour vision may occur.
- On fundoscopy there may be pallor and oedema of the optic disc, and 'cotton-wool' patches and (less uncommonly) small haemorrhages in the retina — these features are usually seen following loss of vision.
- Untreated, the second eye is likely to become affected within 1–2 weeks, although it can be affected within 24 hours.
- Neurological features (30% of cases)
- Mononeuropathy or polyneuropathy of arms or legs
- Transient ischaemic attack or stroke in the distribution of the carotid or vertebrobasilar arteries (less common)
- Upper cranial nerve palsies
- Peripheral arthritis and distal swelling with pitting oedema (25% of cases)
- Respiratory tract symptoms, for example, cough, sore throat, and hoarseness (10% of cases)
Management
- Giant cell arteritis (GCA) is a medical emergency.
- Urgently refer all people with suspected GCA for specialist evaluation (usually by a rheumatologist) on the same working day if possible.
- If there is visual loss (transient or permanent), arrange an urgent (same day) assessment by an ophthalmologist.
- Immediately treat people with suspected GCA with corticosteroids:
- The standard initial dose for active GCA without visual symptoms is 40–60 mg oral prednisolone per day.
- GCA patients with acute or intermittent visual loss may initially be given 500 mg–1 g intravenous methylprednisolone daily for up to 3 consecutive days before commencing oral prednisolone therapy. If intravenous therapy is not immediately possible, this should not delay initiation of oral prednisolone.
- Document the person's symptoms, signs, and level of function both before and after the onset of the condition to use as a baseline to compare response to treatment.
- Consider arranging the following tests and send the results to the specialist to help establish the diagnosis (do not to delay referral while awaiting the results):
- Liver function tests – about a third of people have mildly elevated liver function test results, particularly for alkaline phosphatase.
- Full blood count — normochromic normocytic anaemia and an elevated platelet count are common.
- C-reactive protein (CRP) — the CRP level is typically elevated and may be a more sensitive indicator of inflammation in some people with giant cell arteritis.
- Erythrocyte sedimentation rate (ESR) — the ESR is often greater than 50 mm/hour. However, the ESR may be normal at presentation and even during a flare of disease activity.
- Further investigation:
- Temporal artery biopsy is the definitive test, which all patients with suspected GCA should undergo. The biopsy is performed on the side with abnormal clinical findings (if present). However, if the biopsy on one side is normal, a second biopsy on the contralateral side should be performed. An adequate length of temporal artery (3 to 5 cm) should be obtained because inflammatory lesions may be present in a segmental fashion. Treatment should not be delayed while waiting for the biopsy to be performed. After several weeks of glucocorticoid therapy, temporal artery biopsy may still yield a diagnosis of arteritis.
- Temporal artery biopsy is less helpful in patients with large-vessel GCA and may be negative in up to 50% of these patients. If large-vessel involvement is suspected, this may be diagnosed by conventional angiography, magnetic resonance angiography, or computed tomographic scan.
Complications
- Vision loss
- Total or partial loss of vision may occur in up to 20% of people with giant cell arteritis
- Once vision is lost, there is little chance of recovery, even with treatment. In this situation, treatment is given to prevent further visual deterioration and involvement of the other eye.
- Vision loss is rare once treatment with high dose corticosteroids has commenced.
- Large artery complications
- Large artery complications include aortic aneurysm, aortic dissection, large artery stenosis, and aortic regurgitation.
- Cardiovascular disease
- Cardiovascular disease (such as myocardial infarction, heart failure, stroke, and peripheral arterial disease) is more common in people with giant cell arteritis.
- Other complications
- Peripheral neuropathy
- Depression
- Confusion and encephalopathy (in about 30% of people)
- Deafness
- Complications of long-term corticosteroid treatment (for example weight gain, bruising, osteoporosis and fractures, and diabetes)
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- Biochemistry
- Blood Gases
- Haematology
| Biochemistry | Normal Value |
|---|---|
| Sodium | 135 – 145 mmol/l |
| Potassium | 3.0 – 4.5 mmol/l |
| Urea | 2.5 – 7.5 mmol/l |
| Glucose | 3.5 – 5.0 mmol/l |
| Creatinine | 35 – 135 μmol/l |
| Alanine Aminotransferase (ALT) | 5 – 35 U/l |
| Gamma-glutamyl Transferase (GGT) | < 65 U/l |
| Alkaline Phosphatase (ALP) | 30 – 135 U/l |
| Aspartate Aminotransferase (AST) | < 40 U/l |
| Total Protein | 60 – 80 g/l |
| Albumin | 35 – 50 g/l |
| Globulin | 2.4 – 3.5 g/dl |
| Amylase | < 70 U/l |
| Total Bilirubin | 3 – 17 μmol/l |
| Calcium | 2.1 – 2.5 mmol/l |
| Chloride | 95 – 105 mmol/l |
| Phosphate | 0.8 – 1.4 mmol/l |
| Haematology | Normal Value |
|---|---|
| Haemoglobin | 11.5 – 16.6 g/dl |
| White Blood Cells | 4.0 – 11.0 x 109/l |
| Platelets | 150 – 450 x 109/l |
| MCV | 80 – 96 fl |
| MCHC | 32 – 36 g/dl |
| Neutrophils | 2.0 – 7.5 x 109/l |
| Lymphocytes | 1.5 – 4.0 x 109/l |
| Monocytes | 0.3 – 1.0 x 109/l |
| Eosinophils | 0.1 – 0.5 x 109/l |
| Basophils | < 0.2 x 109/l |
| Reticulocytes | < 2% |
| Haematocrit | 0.35 – 0.49 |
| Red Cell Distribution Width | 11 – 15% |
| Blood Gases | Normal Value |
|---|---|
| pH | 7.35 – 7.45 |
| pO2 | 11 – 14 kPa |
| pCO2 | 4.5 – 6.0 kPa |
| Base Excess | -2 – +2 mmol/l |
| Bicarbonate | 24 – 30 mmol/l |
| Lactate | < 2 mmol/l |
