QuestionSBA
QuestionChoice
300mg of aspirin would be the treatment of choice if this patient were suspected of having a transient ischaemic attack (TIA); however, whilst TIAs can present with blindness, they do not usually cause headache and would not explain the scalp or jaw pain
32719
b
Stat dose 300mg aspirin
QuestionChoice
Low dose amitriptyline may be used in neuropathic pain and migraine prophylaxis; however, whilst migraine can cause headache and visual loss/disturbance in some cases, it is unlikely for a patient to have a new diagnosis of migraine at the age of 63 and therefore other causes should be considered
32720
c
Start 10mg amitriptyline
QuestionChoice
This history is suspicious for temporal arteritis, aka giant cell arteritis (GCA). As she has experienced vision loss, it is important to start high dose steroid treatment immediately; otherwise, she may have permanent loss. Symptoms of GCA include scalp tenderness, jaw claudication and headache. Patients usually require steroids for a two-year course then have complete remission, and steroids can be reduced once symptoms have resolved. The main cause of death and morbidity in GCA are related to long term steroid use, so risks and benefits of treatment should be considered carefully, and gastric and bone protection should be considered
32718
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Start high dose steroids
QuestionChoice
Thrombolysis with alteplase would be an appropriate treatment for an ischaemic stroke within 4.5 hours of the onset of symptoms. Whilst ischaemic stroke can cause blindness, it would not usually cause headaches and would not explain the scalp or jaw pain
32722
e
Stat dose 900mcg/kg altepalase
QuestionChoice
Carbemazipine is the first line treatment for trigeminal neuralgia. This would be in the differential for unilateral facial pain; however, it would not account for the blindness, and the pain she describes does not sound neuropathic
32721
d
Start 100mg carbamazepine
Concept
Chapter
# Summary

Giant cell arteritis (GCA) is a form of vasculitis which affects medium and large arteries. It classically affects the branches of the external carotid artery and the ophthalmic artery, which is often referred to as temporal arteritis. Key signs and symptoms include a temporal headache, jaw claudication, amaurosis fugax, thickening and tenderness of the temporal artery, and scalp tenderness. Systemic symptoms and features of polymyalgia rheumatica are common. The diagnosis is clinical in the first instance, and patients with visual symptoms should be treated immediately with high-dose steroids, however supportive investigations include full blood count, CRP and ESR. Confirmation of the diagnosis is with vascular ultrasound and temporal artery biopsy. Management is with high-dose steroids, with steroid sparing agents considered in patients who relapse when steroids are weaned.

# Definition

Giant cell arteritis (GCA) is a large vessel vasculitis which commonly affects the extracranial external carotid artery branches. Involvement of other vessels may occur, with patients presenting atypically with systemic symptoms and aortic involvement rather than temporal arteritis.

# Epidemiology

- GCA is the most common primary vasculitis 
- Age is an important risk factor - GCA is rare in the under 50s, with cases peaking in those aged 70-79
- Women are 2-3x more likely to be affected than men
- Northern European descent is also a risk factor

# Signs and Symptoms

**Symptoms include:**

- Headache - usually temporal but may be generalised, occipital or parietal; present in 2/3 of people with GCA
- Jaw or tongue claudication (pain on chewing food)
- Amaurosis fugax (transient monocular blindness, often described as a dark curtain descending vertically)
- Diplopia
- Changes to colour vision
- Fatigue
- Anorexia and weight loss
- Depression
- Features of polymyalgia rheumatica e.g. pain and stiffness of shoulders and pelvic girdle

**Signs include:**

- Tenderness, thickening or nodularity of the temporal artery
- Pulsation of the temporal artery may be reduced or absent
- Scalp tenderness or necrosis
- Visual field defect
- Low-grade fever
- On fundoscopy: pallor and oedema of the optic disc, "cotton-wool" patches and retinal haemorrhages
- Involvement of extracranial vessels may be associated with arterial bruits, difference in blood pressure between arms and decreased arterial pulses

# Differential Diagnosis

- **Other causes of headaches** e.g. tension headaches, migraines - lack associated features e.g. jaw claudication, less likely to be new in an older patient
- **Acute angle closure glaucoma** leads to visual loss and headaches, eye will be painful and red and feel hard on palpation
- **Shingles** leads to pain and systemic features may be present, however a rash typically appears over the affected area within a few days
- **Transient ischaemic attack** can cause amaurosis fugax, other features such as headache are not usually present


# Investigations

**Bedside tests:**

- Urgent ophthalmological assessment for patients with visual symptoms 

**Blood tests:**

- **ESR** and **CRP** - raised in GCA
- **Full blood count** often shows an elevated platelet count and a normochromic normocytic anaemia
- Baseline bloods for liver and renal function (**LFTs** and **U&Es**) and a **bone profile** for calcium should be considered to screen for alternative diagnoses e.g. other vasculitides
- Bloods to screen for increased risk of complications with steroids (such as diabetes or osteoporosis) include an **HbA1c**, **vitamin D** and **thyroid function tests** 

**Imaging tests:**

- **Doppler ultrasonography** of the temporal and axillary arteries can be used as a confirmatory diagnostic test
- **Halo sign** refers to hypoechoic wall thickening of the artery imaged
- This disappears with steroid treatment
- Stenosis of the vessel may also be visualised
- **MRI brain** with vessel wall imaging is an alternative
- GCA is supported by findings of mural inflammation in the superficial temporal arteries
- MRI can also be used to look for complications such as ischaemic stroke as well as involvement of other arteries
- **FDG-PET scanning** can be useful to rule out differential such as malignancy or infection
- In GCA there will be uptake in affected arteries
- It can be particularly useful to demonstrate involvement of other arteries e.g. the aorta
- A **DEXA** scan for baseline bone density should be done for patients starting long-term steroids (due to the risk of osteoporosis)

**Invasive tests:**

- **Temporal artery biopsy**
- This is the definitive investigation for GCA
- Typical histological features include granulomatous inflammation of arteries with inflammatory cells including multinucleated giant cells
- Inflammation is often segmental with "skip lesions" i.e. normal parts of the artery in between areas of inflammation, and so false negative biopsies can occur
- Biopsies may remain positive for several weeks after starting steroids

# Management

- Patients with visual loss usually require **IV steroid treatment** e.g. pulsed methylprednisolone
- All other patients should be treated with **high dose oral steroids**, usually 40-60 mg prednisolone once a day
- **Safety netting** patients to seek urgent medical attention if they develop visual symptoms or a relapse on steroids is key
- Patients seen in primary care should be **referred urgently to rheumatology** for confirmation of the diagnosis and ongoing management
- **Steroids are tapered** gradually once symptoms and raised inflammatory markers have resolved
- **Steroid sparing agents** such as methotrexate or tocilizumab (an anti-IL6 biologic) may be added to help with steroid tapering e.g. in patients with recurrent relapses or at increased risk of side effects with steroids
- All patients should be assessed for risk of steroid side effects:
- Consider gastric protection with a proton pump inhibitor
- Consider bone protection e.g. vitamin D and calcium supplementation, bisphosphonates
- Monitor for steroid-induced diabetes, hypertension and glaucoma
- Advise on immunosuppression e.g. avoiding contact with patients with diseases such as chickenpox if not immune
- Give patients a blue steroid card and advise them not to suddenly stop steroids due to the risk of adrenal crisis
- Aspirin is currently **not** recommended for adjunctive treatment of GCA 

# Complications

- Loss of vision - may occur in up to 30% of people with GCA (either total or partial)
- Scalp necrosis
- Ischaemic stroke
- Aortic aneurysms - in 10-20% of people with GCA, most commonly in the thoracic aorta
- Aortic dissection and aortic regurgitation are less common than aneurysms
- Stenosis of large arteries
- Increased risk of cardiovascular disease including stroke, myocardial infarction and peripheral arterial disease
- Side-effects from long-term steroids e.g. osteoporosis and fragility fracture, weight gain, diabetes, gastric ulcers

# Prognosis

- Most patients respond rapidly to steroids - failure to respond should prompt investigations for an alternative diagnosis
- Up to 50% of people will experience relapsing disease however
- Most patients require at least 1-2 years of steroid treatment, with an increased risk of relapse if steroids are stopped within a year of diagnosis

# NICE Guidelines

[NICE CKS - Giant Cell Arteritis](https://cks.nice.org.uk/topics/giant-cell-arteritis/)

# References

[British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis](https://academic.oup.com/rheumatology/article/59/3/e1/5714024)

[Radiopaedia - Giant Cell Arteritis](https://radiopaedia.org/articles/giant-cell-arteritis?lang=gb)

[Patient UK - Giant Cell Arteritis](https://patient.info/doctor/giant-cell-arteritis-pro)
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Giant cell arteritis (GCA) is an inflammatory condition affecting large blood vessels, often presenting with symptoms like headache and vision changes; it requires prompt treatment with high-dose steroids to prevent complications such as vision loss and reduce inflammation.
A 63-year-old female presents to Accident and Emergency with sudden right-sided blindness associated with a headache. She has never had issues with headaches before. On further questioning, she reveals that she has tenderness on brushing her hair and has been to the dentist recently with toothache but did not need any dental work done.

What should your initial management be?
a