Giant Cell Arteritis

Giant cell arteritis is a systemic vasculitis of the medium and large arteries. It typically presents with symptoms affecting the temporal arteries and is also known as temporal arteritis.

There is a strong link with polymyalgia rheumatica. The patients at higher risk are white females over 50.

The key complication of giant cell arteritis is vision loss. This is often irreversible. High dose steroids are used immediately once a diagnosis is suspected to prevent the development of progression of vision loss.

 

Symptoms

The main presenting feature is a headache:

  • Severe unilateral headache typically around temple and forehead
  • Scalp tenderness my be noticed when brushing hair
  • Jaw claudication
  • Blurred or double vision
  • Irreversible painless complete sight loss can occur rapidly

 

There may be associated systemic symptoms such as:

  • Fever
  • Muscle aches
  • Fatigue
  • Weight loss
  • Loss of appetite
  • Peripheral oedema

 

Diagnosis

A definitive diagnosis is based on:

  • Clinical presentation
  • Raised ESR: usually 50 mm/hour or more
  • Temporal artery biopsy findings

 

TOM TIP: Multinucleated giant cells are found on the temporal artery biopsy. This is what gives rise to the giant cell arteritis name. This is worth remembering for your exams as it is a popular question.

 

Additional Investigations

  • Full blood count may show a normocytic anaemia and thrombocytosis (raised platelets)
  • Liver function tests can show a raised alkaline phosphatase
  • C reactive protein is usually raised
  • Duplex ultrasound of the temporal artery shows the hypoechoic halo sign

 

Initial Management

Steroids:

Start steroids immediately before confirming the diagnosis to reduce the risk of permanent sight loss. Start 40-60mg prednisolone per day. 60mg is given depending where there are jaw claudication or visual symptoms. Review the response to steroids within 48 hours. There is usually a rapid and significant response to treatment.

 

Other medications:

  • Aspirin 75mg daily decreases visual loss and strokes
  • Proton pump inhibitor (e.g. omeprazole) for gastric prevention while on steroids

 

Referrals:

  • Vascular surgeons for a temporal artery biopsy in all patients with suspected GCA
  • Rheumatology for specialist diagnosis and management
  • Ophthalmology review as an emergency same day appointment if they develop visual symptoms

 

Ongoing Management

Once the diagnosis is confirmed they will need to continue high dose steroids (40-60mg) until the symptoms have resolved. They then need to slowly wean off the steroids. This can take several years. This is a similar process to managing polymyalgia rheumatica.

There are additional measures for patients on steroids that can be remembered by the mnemonic “Don’t STOP”:

  • DON’T – Don’t stop taking steroids abruptly. There is a risk of adrenal crisis.
  • S – Sick Day Rules.
  • T – Treatment Card.
  • O – Osteoporosis prevention with bisphosphonates and supplemental calcium and vitamin D.
  • P – Proton pump inhibitor for gastric protection.

 

Complications

Early neuro-ophthalmic complications:

  • Vision loss
  • Cerebrovascular accident (stroke)

 

Late:

  • Relapses of the condition are common
  • Steroid related side effects and complications
  • Cerebrovascular accident (stroke)
  • Aortitis leading to aortic aneurysm and aortic dissection

 

Last updated April 2019