What are the signs and symptoms of giant cell arteritis?

Signs and Symptoms of Giant Cell Arteritis

Giant cell arteritis (GCA) typically presents with headache, scalp tenderness, jaw claudication, and constitutional symptoms in patients over 50 years of age, with visual symptoms representing the most serious complications that require urgent treatment. 1

Common Clinical Manifestations

Cranial Symptoms

• Headache - often severe and persistent, typically localized in the temporal area 1
• Scalp tenderness - sensitivity when touching the scalp 1
• Jaw claudication - pain in the jaw when chewing 1
• Temporal artery abnormalities - tenderness, thickening, nodularity, and/or reduced or absent pulsation 1
• Tongue claudication - less common but specific 1

Visual Symptoms

• Amaurosis fugax (transient visual loss) - occurs in approximately 20% of patients 1
• Permanent partial or complete visual loss - occurs in approximately 10% of patients 1, 2
• Diplopia (double vision) - due to ischemia of ocular muscles 3, 4
• Visual field defects 1, 2
• Anterior ischemic optic neuropathy - most common cause of permanent visual loss 4, 2

Constitutional Symptoms

• Fever/pyrexia - typically low-grade 1, 5
• Fatigue and malaise 1, 5
• Weight loss (>2 kg) 1, 5
• Night sweats 1, 5
• Anorexia 5, 4

Polymyalgia Rheumatica

• Present in many GCA patients 1, 6
• Characterized by pain and stiffness in the shoulder and hip girdles 1

Large Vessel Involvement

• Limb claudication 1
• Reduced pulses/blood pressure in upper limbs 1
• Bruits (particularly in the axilla) 1
• Arterial stenosis or aneurysms 1

Laboratory Findings

• Elevated erythrocyte sedimentation rate (ESR) - typically >40 mm/h, often >100 mm/h 1
• Elevated C-reactive protein (CRP) 1, 5
• Thrombocytosis (platelet count >400 × 10³/μL) 1
• Anemia 5, 4

Less Common Manifestations

• Respiratory symptoms - chronic cough (typically harsh and nonproductive) occurs in <10% of cases 1
• Sore throat and hoarseness 1
• Tenderness of cervical structures 1
• Uveitis - rare presentation 7
• Fatigable ptosis - atypical presentation 3

Diagnostic Significance

• Jaw claudication (positive LR 4.90) is one of the most specific symptoms 1
• Temporal artery thickening (positive LR 4.70) and loss of pulse (positive LR 3.25) are highly suggestive physical findings 1
• ESR >100 mm/h (positive LR 3.11) strongly supports the diagnosis 1
• Absence of elevated ESR >40 mm/h (negative LR 0.18) makes GCA less likely 1

Warning Signs Requiring Urgent Treatment

• Any visual symptoms in a patient with suspected GCA require immediate treatment with high-dose glucocorticoids (40-60 mg/day prednisone) to prevent permanent vision loss 1, 2
• Patients with visual symptoms have a high risk (up to 50%) of developing visual loss in the fellow eye within days if left untreated 2
• Headache with constitutional symptoms in patients over 50 years should raise immediate suspicion for GCA 1

Clinical Pearls

• No single symptom or sign is pathognomonic for GCA, but combinations of symptoms significantly increase diagnostic probability 1
• Visual complications represent the most feared manifestation of GCA and require urgent intervention 6, 2
• "Troublesome cough of unknown etiology" combined with headaches in persons >50 years should raise suspicion for GCA 1
• GCA can sometimes present atypically without the classic cranial symptoms, leading to diagnostic delays 6, 3