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Giant Cell Arteritis (GCA)

GCA occurs primarily in patients >50 years of age. The incidence increases with age, and GCA is almost 10 times more common among patients in their 80s than in patients aged 50 to 60 years. GCA is two to three times more common among women than men.

Most patients will have one of five presentations:

  • Some 20% of patients have cranial symptoms with superficial headache, scalp tenderness, jaw and tongue claudication, and rarely scalp necrosis, diplopia, or blindness.

  • Approximately 40% of patients have polymyalgia rheumatica (PMR) with pain and stiffness of proximal muscle groups, such as the neck, shoulders, hips, and thighs. Muscle symptoms are usually symmetric and the shoulders are most commonly involved. Note that 10% to 20% of patients presenting with PMR develop GCA on follow-up.

  • Some 20% of patients have both cranial and PMR symptoms. About 40% to 50% of patients with GCA have PMR symptoms.

  • About 15% of patients have fever and systemic symptoms without any localized symptoms. Patients can present with a fever of unknown origin (FUO). GCA is the cause in 15% of elderly patients with an FUO.

  • Some 5% of patients have other symptoms, such as cough, claudication (upper > lower extremity), or synovitis.

-->GCA should be suspected in individuals aged >50 to 60 years with new onset of unexplained pain above the neck (headache, jaw claudication, other areas mimicking sinusitis/ear pain), unexplained fever, or PMR.

GCA facts and clinic

  • Large vessel vasculitis, especially of the thoracic aorta and its branches
  • polymyalgic pain shoulder/hip girdle, neck stiffness
  • Increased CRP/increased BSR
  • Peripheral arthritis/RS3PE syndrome
  • Systemic symptoms (fever, weight loss (>2 kg), night sweats, depression) Headache
  • Sensitive/painful scalp Vascular swelling/pain, auscultation sound Claudicatio masticatoris/pain and claudication of the tongue
  • Blurred vision: Amaurosis fugax, double vision, blindness
  • (specific findings: AION, central ocular muscle paralysis, occlusion of arterial retinal vessels and/or choroidal ischemia)
  • difficulty swallowing / dysphagia
  • ischemia of the extremities, missing or asymmetric pulses, blood pressure difference, Raynaud's phenomenon
  • Aortic Insufficiency


Complications for Different Vascular Distributions in Giant Cell Arteritis:

Ophthalmic

Blindness

Subclavian

Absent pulses

Renal

Hypertension

Coronary

Angina pectoris

Carotid

Stroke

Vertebral

Dizziness, stroke

Iliac

Claudication

Mesenteric

Abdominal pain

There have been many instances that support the hypothesis that corticosteroid therapy can prevent catastrophic events in patients with temporal arteritis. Sudden blindness and other stroke-like events have occasionally been reversed in 33% of patients by institution of high-dose corticosteroid therapy (1 g of intravenous methylprednisolone daily for 3 days) if started within 24 hours of the event. Consider Tocilizumab-therapy.

The risk of death from GCA appears to be increased (threefold) within the first 4 months of starting therapy. Patients typically die of vascular complications, such as stroke or myocardial infarction, supporting the use of low-dose aspirin in patients diagnosed with GCA. BP must be controlled and the patient should stop smoking.

Surgery is considered when the aneurysm enlarges to greater than 5 cm or dissects.


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