What are the common presenting symptoms of giant cell arteritis in patients over 50 years old, especially women?

Giant Cell Arteritis: Clinical Presentation and Symptoms

Giant cell arteritis (GCA) typically presents in patients over 50 years old with new-onset headache (occurring in 66-90% of cases), jaw claudication (the most specific symptom), scalp tenderness, and visual disturbances, with women affected 2-3 times more often than men. 1, 2

Cardinal Cranial Symptoms

Headache

• New-onset headache is the most common presenting symptom, occurring in two-thirds of patients and up to 90% of biopsy-confirmed cases 1, 2
• The headache is typically localized to the temporal region and represents a new pattern for the patient 3
• However, headache has limited diagnostic specificity once GCA is suspected, as it occurs frequently in both GCA-positive and GCA-negative patients 1

Jaw Claudication

• Jaw claudication is the most specific symptom with a positive likelihood ratio of 4.90, present in approximately 50% of patients 1, 2
• Characterized by pain during chewing that resolves with rest 2
• This symptom strongly increases the probability of GCA when present 1

Visual Symptoms

• Visual disturbances occur in 20% of patients before permanent vision loss, including transient vision loss (amaurosis fugax), diplopia, or blurred vision 2
• Anterior ischemic optic neuropathy (AION) occurs in 23.9% of cases (positive likelihood ratio 2.15) 1
• Untreated GCA carries a 14-50% risk of permanent, irreversible blindness, making this the most feared complication 1, 2

Scalp Tenderness

• Scalp sensitivity has a positive likelihood ratio of 3.14, making it a highly suggestive finding 1, 2
• Patients may report pain when combing hair or resting their head on a pillow 2

Physical Examination Findings

Temporal Artery Abnormalities

• Temporal artery thickening has the highest positive likelihood ratio (4.70) among physical findings 1, 2
• Loss of temporal artery pulse yields a positive likelihood ratio of 3.25 1, 2
• Temporal artery tenderness has a positive likelihood ratio of 1.93 1
• Any temporal artery abnormality (thickening, loss of pulse, or tenderness) has a positive likelihood ratio of 3.14 1

Systemic and Constitutional Symptoms

Common Systemic Features

• Constitutional symptoms including fever, weight loss (≥2 kg), fatigue, malaise, and night sweats are common but have limited diagnostic specificity 1, 2, 4
• These nonspecific symptoms may dominate the clinical presentation and delay diagnosis 3

Polymyalgia Rheumatica

• Polymyalgia rheumatica symptoms (proximal muscle pain and stiffness in shoulders, neck, and pelvic girdle) frequently coexist with GCA 2, 4, 5
• PMR and GCA occur together more frequently than expected by chance 4

Large Vessel Involvement

Extracranial Manifestations

• Limb claudication carries the highest positive likelihood ratio (6.01) among all symptoms, indicating severe ischemic complications from large vessel stenosis 2
• Absent pulses and blood pressure differences between upper limbs may be present 1, 2
• Auscultation may reveal bruits, especially over the axillary region 2

Less Common Ischemic Complications

• Stroke, cranial nerve palsies, and scalp necrosis can occur 6
• Large-vessel aneurysms and vascular stenoses may develop 6

Laboratory Markers

Inflammatory Markers

• Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are present in more than 95% of cases at diagnosis 2, 6, 7
• ESR >100 mm/h has a positive likelihood ratio of 3.11 1, 2
• The absence of ESR >40 mm/h has a negative likelihood ratio of 0.18, making GCA highly unlikely 1, 2
• The absence of CRP ≥2.5 mg/dL has a negative likelihood ratio of 0.38, strongly arguing against the diagnosis 1, 2

Other Laboratory Findings

• Platelet count >400 × 10³/μL has a positive likelihood ratio of 3.75 1, 2
• Anemia is present in 87.4% of cases but has low specificity (positive likelihood ratio 1.27) 1

Demographic Factors

Age and Sex

• GCA occurs almost exclusively in patients over 50 years of age, with peak incidence at 70-75 years 1, 4, 5
• Age over 70 years increases diagnostic probability; absence of this age has a negative likelihood ratio of 0.48 1, 2
• Women are affected 2-3 times more often than men (ratio 3:2) 2, 4

Ethnicity

• GCA is more frequent in populations of Northern European descent 1, 2

Atypical Presentations and Pitfalls

Diagnostic Challenges

• Although rare (<5% of cases), GCA can present with normal ESR and CRP; high clinical suspicion should still prompt temporal artery biopsy or imaging 2
• Atypical presentations including fever of unknown origin or chronic cough may lead to delayed diagnosis 8
• Respiratory involvement occurs in <10% of cases, with chronic cough being the most frequent respiratory symptom 6, 7

Critical Clinical Caveat

• The most serious complications—blindness and stroke—may occur in the absence of headache, emphasizing the importance of recognizing other symptoms 3
• Classic features like headache and constitutional symptoms have limited use for upgrading or downgrading clinical probability once GCA is suspected, because they occur frequently in both GCA-positive and GCA-negative patients 1

Urgent Management Principle

When GCA is clinically suspected based on the above symptoms, high-dose glucocorticoids (prednisone 40-60 mg daily) must be initiated immediately without awaiting temporal artery biopsy or imaging results, because irreversible vision loss can occur within hours to days. 1, 2