Synonym: temporal arteritis, cranial arteritis
Giant cell arteritis (GCA) is a systemic immune-mediated vasculitis affecting medium-sized and large-sized arteries, particularly the aorta and its extracranial branches.
The aetiology is unknown, but the pathogenesis involves a chronic inflammatory process, predominantly of large arteries.
- GCA occurs in 2.2 per 10,000 patient years in the UK.
- The reported rates for GCA are highest in Northern European countries.
- Women are affected two to three times more often than men.
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- Increased prevalence of PMR and GCA exists in individuals with a European background, and a decreased incidence and prevalence is noted in African Americans.
- Most patients with PMR or GCA present after their sixth decade, and peak incidence occurs in patients aged 60-80 years.
- Genetic factors: PMR and GCA may aggregate in families.
GCA typically presents with recent onset of temporal headache, myalgia, malaise or fever. Typical features may be absent or subtle. GCA should be suspected in anyone over the age of 50 with headache, scalp tenderness, transient visual symptoms or unexplained facial pain.
Acute blindness occurs in up to 20% of patients. Delay in diagnosis and treatment may explain the high incidence of irreversible loss of vision. Jaw or tongue claudication occurs in a minority of cases but indicates a high risk of ischaemic complications.
Symptoms moderately predictive of a positive biopsy result include:
- Jaw claudication (pain comes on gradually during chewing, whereas temporomandibular pain or dental pain is immediate).
- Any abnormality on palpation of the temporal artery (absent pulse, beaded, tender or enlarged).
Other useful predictive features include:
- Temporal headache.
- Scalp tenderness.
- ESR significantly elevated.
The history is usually short, and the most common symptoms include:
- Headache: present in more than 85% of patients. It tends to be of recent onset, or represents a change in character from previous headaches. It is often in the temporal or occipital region, and is described as severe by most patients. It may be worse at night.
- Scalp tenderness - may be pronounced, making simple tasks such as combing hair, or resting the head on a pillow extremely painful.
- Jaw claudication - particularly prominent when the patient is talking or eating, and is present in more than half of patients with temporal arteritis.
- Visual disturbances:
- Due to inflammation of the branches of the ophthalmic artery, leading to ischaemic optic neuritis.
- Occur in around 50% of cases.
- Central retinal artery thrombosis can also occur.
- Visual manifestations include blurred vision, amaurosis fugax, transient or permanent visual loss, or diplopia (due to third, fourth, or sixth cranial nerve palsy).
- These symptoms can occur in the absence of, or before the development of headache.
- If GCA remains untreated, the second eye may become affected within 1-2 weeks.
- Systemic symptoms (similar to those of PMR) include: anorexia, weight loss, fever, sweats, malaise, fatigue and depression. About 50% of patients with GCA have features of PMR: proximal stiffness, soreness and pain.
- Thoracic aorta and aortic root involvement: occurs in about 15%. This is more common in women and younger patients. Thoracic aneurysms can develop as late as 15 years after the diagnosis and successful treatment of GCA.
- Occasionally, symptoms relate to intermittent or persistent brain ischaemia, due to a subclavian steal syndrome (SSS), narrowing of other aortic arch vessels or intracerebral vascular disease. About 1% of cases present with symptoms of brainstem stroke.
- Abdominal aorta involvement can occur, with symptoms of aortic aneurysms and intestinal infarction. Renal involvement is rare.
Examination may be normal but palpation of the temporal artery is often abnormal.
- There may be few signs but there is usually ocular and funduscopic evidence of ischaemic disease present in patients with symptoms of visual loss.
- Temporal arteries may be prominent, beaded, tender, and pulseless but a normal appearance does not exclude the diagnosis.
- Bruits may be heard over the carotid, axillary, or brachial arteries.
- Patients may present with fever.
- Muscles and joints may be tender.
The American College of Rheumatology classification criteria for GCA:
- Age at disease onset: development of symptoms or findings beginning at the age of ≥50 years.
- New headache: new onset of or new type of localised pain in the head.
- Temporal artery abnormality: temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries.
- Elevated ESR: ESR ≥50 mm/hour.
- Abnormal artery biopsy: biopsy specimen with artery showing vasculitis characterised by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells.
A patient is said to have GCA if at least three of these five criteria are present. The presence of any three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.
- Tension headache
- Trigeminal neuralgia
- Takayasu's arteritis
- Polyarteritis nodosa
Once GCA is suspected, patients should be referred urgently for temporal artery biopsy, which needs to be done within two weeks of starting steroids.
- Elevation in the acute phase reactants: 4% of patients have a normal ESR; 83% have a rate above 50 mm/hour. A normal ESR does not therefore exclude the diagnosis.
- CRP can sometimes be elevated in the presence of a normal ESR.
- Normocytic normochromic anaemia and thrombocytosis are common.
- Auto-antibody and complement levels are normal. Cryoglobulins and monoclonal immunoglobulins are absent. Muscle enzyme levels - eg, creatine kinase and aldolase, are normal.
- LFTs, especially the alkaline phosphatase, may be elevated.
- Temporal artery biopsy:
- The sensitivity of temporal biopsy is not known, but has been estimated to be 87%.
- Biopsy should be taken on the symptomatic side.
- Colour duplex ultrasonography has been shown to be relatively accurate for diagnosing GCA.
16-21% of patients with PMR have GCA on temporal artery biopsy, and symptoms of PMR are present in 40-60% of patients with GCA.
Urgent referral for specialist evaluation is recommended for all patients with suspected GCA but this should not delay promptly starting high-dose steroid treatment.
If temporal artery biopsy is negative (either due to the presence of skip lesions or suboptimal biopsy), patients should be managed as having GCA if there is a typical clinical and laboratory picture and response to glucocorticosteroids, or typical findings on ultrasound, or ischaemic complications typical of GCA (such as anterior ischaemic optic neuritis).
- Once the diagnosis is suspected, treat with high-dose corticosteroid immediately:
- 40 mg prednisolone daily unless the patient has ischaemic symptoms (jaw or tongue claudication, or visual symptoms).
- Claudication symptoms: give 60 mg prednisolone daily.
- If the patient has visual symptoms, admit for treatment with intravenous methylprednisolone.
- Once symptoms and abnormal test results resolve, the dose can be reduced in 10 mg steps each two weeks to 20 mg, then in 2.5 mg steps.
- Flare-ups and relapses usually respond to corticosteroid increases to the level at which symptoms previously were controlled. Protracted courses of therapy are often necessary.
- Once the diagnosis is suspected, treat with high-dose corticosteroid immediately:
- Low-dose aspirin:
- Start aspirin 75 mg daily unless there are contra-indications - eg, active peptic ulceration or a bleeding disorder.
- Start gastroprotection with a proton pump inhibitor in view of added risk of peptic ulceration with high-dose steroids and aspirin.
- Low-dose aspirin has been shown to decrease the rate of visual loss and strokes in patients with GCA.
Other treatments: although steroids remain the cornerstone of therapy for GCA, patients with long-standing, recalcitrant disease may be considered for treatment with methotrexate, azathioprine or tumour necrosis factor-alpha inhibitors.
- Osteoporosis prophylaxis is required for patients on long-term steroid treatment.
- Loss of vision.
- Aneurysms, dissections and stenotic lesions of the aorta and its major branches.
- Central nervous system disease: seizures, cerebral vascular accidents. SSS and brain ischaemia. Intracranial vessels are involved only rarely. Peripheral nerve involvement is also rare.
- Steroid-related complications are common; morbidity from steroid therapy is often worse than the underlying disease. Potential steroid-related complications include osteoporosis, corticosteroid myopathy, bruising, emotional symptoms (eg, insomnia, restlessness, hypomania, depression), hypertension, diabetes, elevated cholesterol and fluid retention.
- Visual damage is often irreversible. Partial or complete loss of vision occurs in about 15-20% of patients. Blindness in both eyes is rare.
- Patients experience relief with treatment, but spontaneous relapses are common and unpredictable within the first years of the disease.
- Most patients are able to stop taking steroids by two years.
- Factors that predict the need for prolonged therapy and increased relapse risk are older age at diagnosis, female sex, higher baseline ESR and initial rapid reduction of prednisolone dose.
Further reading & references
- Seetharaman M et al; Giant Cell Arteritis, Medscape, May 2012
- Kale N, Eggenberger E; Diagnosis and management of giant cell arteritis: a review. Curr Opin Ophthalmol. 2010 Nov;21(6):417-22. doi: 10.1097/ICU.0b013e32833eae8b.
- Salvarani C, Cantini F, Hunder GG; Polymyalgia rheumatica and giant-cell arteritis. Lancet. 2008 Jul 19;372(9634):234-45.
- Hassan N, Dasgupta B, Barraclough K; Giant cell arteritis. BMJ. 2011 May 23;342:d3019. doi: 10.1136/bmj.d3019.
- Liozon E, Ouattara B, Rhaiem K, et al; Familial aggregation in giant cell arteritis and polymyalgia rheumatica: a comprehensive literature review including 4 new families. Clin Exp Rheumatol. 2009 Jan-Feb;27(1 Suppl 52):S89-94.
- BSR and BHPR guidelines for the management of giant cell arteritis, British Society for Rheumatology (March 2010)
- Hunder GG, Bloch DA, Michel BA, et al; The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990 Aug;33(8):1122-8.
- Ball EL, Walsh SR, Tang TY, et al; Role of ultrasonography in the diagnosis of temporal arteritis. Br J Surg. 2010 Dec;97(12):1765-71. doi: 10.1002/bjs.7252.
- Unwin B, Williams CM, Gilliland W; Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician. 2006 Nov 1;74(9):1547-54.
- Giant cell arteritis, Prodigy (May 2009)
- Nesher G, Berkun Y, Mates M, et al; Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum. 2004 Apr;50(4):1332-7.
- Pipitone N, Salvarani C; Improving therapeutic options for patients with giant cell arteritis. Curr Opin Rheumatol. 2008 Jan;20(1):17-22. doi: 10.1097/BOR.0b013e3282f31769.
|Original Author: Dr Colin Tidy||Current Version: Dr Colin Tidy||Peer Reviewer: Dr Adrian Bonsall|
|Last Checked: 11/02/2013||Document ID: 2194 Version: 23||© EMIS|
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