What are the signs and symptoms of Giant Cell Arteritis (GCA)?

Signs and Symptoms of Giant Cell Arteritis

Giant Cell Arteritis (GCA) presents with a constellation of cranial and systemic manifestations, with headache being the most common symptom, but visual disturbances representing the most serious complication that requires urgent treatment to prevent irreversible vision loss. 1

Cranial Manifestations

Common Cranial Symptoms

• Headache: Often severe and typically localized to the temporal area (bitemporal); the most common symptom 1
• Scalp tenderness: Sensitivity when touching the scalp, particularly over temporal regions 1
• Temporal artery abnormalities: Including:
  • Thickening of the temporal artery
  • Tenderness along the artery
  • Decreased or absent pulse in the temporal artery 1
• Jaw claudication: Pain in the jaw muscles when chewing or talking 1, 2

Visual Manifestations

• Amaurosis fugax (temporary vision loss): Occurs in approximately 20% of patients 1
• Permanent visual loss: Occurs in 10-18% of patients; often irreversible if treatment is delayed 1, 3
• Diplopia: Double vision due to cranial nerve involvement 1

Less Common Cranial Features

• Scalp necrosis: Rare but serious ischemic complication 4
• Tongue necrosis: Uncommon manifestation 5
• Lip necrosis: Rare presentation 5
• Cranial nerve palsy: Neurological complication 1
• Stroke: Can occur due to involvement of cerebral vessels 1

Systemic Manifestations

Constitutional Symptoms

• Low-grade fever: Common non-specific manifestation 1
• Malaise: General feeling of unwellness 1
• Weight loss: Often significant and unintentional 1
• Fatigue: Persistent tiredness 5

Polymyalgia Rheumatica Features

• Polymyalgic symptoms: Pain and stiffness in the shoulders, neck, and hip girdle 1
• Morning stiffness: Particularly affecting proximal muscles 2

Respiratory Symptoms

• Chronic cough: Harsh, nonproductive cough occurring in <10% of cases; may be associated with:
  • Sore throat
  • Hoarseness
  • Tenderness of cervical structures 1

Vascular Manifestations

• Limb claudication: Due to arterial stenosis 1
• Absent pulses: In affected extremities 1
• Large vessel involvement: Can lead to aortic aneurysms and stenosis of major branches 1

Laboratory Findings

• Elevated erythrocyte sedimentation rate (ESR): Typically >50 mm/hr, often >100 mm/hr; present in >95% of cases 1
• Elevated C-reactive protein (CRP): Present in >95% of cases 1
• Anemia: Common finding 1
• Thrombocytosis: Platelet count often >400 × 10³/μL 1

Diagnostic Considerations

The diagnosis of GCA should be strongly considered in patients over 50 years of age presenting with new-onset headache, particularly when accompanied by visual disturbances, jaw claudication, or polymyalgia symptoms 1. A combination of clinical features, laboratory findings, and either temporal artery biopsy or vascular imaging is typically required for definitive diagnosis 1.

Clinical Pitfalls to Avoid

1. Delayed recognition of visual symptoms: Vision loss can become irreversible if treatment is not initiated promptly 3, 5
2. Overlooking GCA in patients with atypical presentations: Such as isolated respiratory symptoms or fever of unknown origin 1
3. Relying solely on ESR: While typically elevated, some patients may initially present with normal inflammatory markers 3
4. Failing to consider GCA in patients with troublesome cough: Particularly in those >50 years with headaches 1
5. Missing large vessel involvement: Which may present with limb claudication rather than classic cranial symptoms 1

Early recognition and prompt treatment with high-dose glucocorticoids is essential to prevent irreversible complications, particularly vision loss 1, 3.