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The basic pathology of this very important disease complex is giant cell arteritis (synonyms: temporal arteritis; cranial arteritis). The clinical syndromes are polymyalgia rheumatica and temporal arteritis.
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Clinical features of polymyalgia rheumatica
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Pain and stiffness in proximal muscles of shoulder and pelvic girdle, cervical spine
Symmetrical distribution
Typical ages 60–70 yrs (rare <50)
Both sexes: more common in women
Early morning stiffness lasting >45 mins.
May be systemic symptoms: weight loss, malaise, anorexia, fever
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Clinical features of temporal arteritis (TA)
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New headache—unilateral, throbbing
Visual symptoms, e.g. diplopia
Temporal artery tenderness
Loss of pulsation of temporal artery
Jaw claudication
Biopsy of artery (5 cm) is diagnostic
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DxT: fatigue/malaise + headache + jaw claudication → TA
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No specific test for polymyalgia rheumatica
ESR—extremely high, typically >40, may be around 100; ↑ CRP
Mild anaemia (normochromic, normocytic)
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Treatment (refer any patient with GCA immediately)
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Starting dose: temporal arteritis usually 1 mg/kg, 60 mg average; polymyalgia rheumatica 15 mg
After 2–4 wks, taper down gradually (max. 10% wkly) to min. effective dose (often <5 mg/d) according to the clinical response and the ESR. Aim for treatment for 2 yrs: relapses are common.
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Azathioprine or methotrexate can be used as steroid-sparing agents.