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Temporal Arteritis (Giant Cell Arteritis)

Temporal Arteritis, also known as Giant Cell Arteritis (GCA), is a serious inflammatory condition that affects the large and medium-sized arteries, most commonly those in the head, especially the temporal arteries located on the sides of the head. It is considered a medical emergency due to the risk of permanent vision loss.

Causes:

The exact cause of Temporal Arteritis is unknown, but it is classified as an autoimmune disease, where the body’s immune system mistakenly attacks healthy blood vessel walls, causing inflammation and swelling. Factors that may contribute include:

  • Age: GCA almost exclusively affects people over the age of 50, with the average age of onset around 70.
  • Sex: Women are about two to three times more likely to develop GCA than men.
  • Genetics: There appears to be a genetic predisposition, as GCA is more common in people of Northern European descent.
  • Polymyalgia Rheumatica (PMR): A significant percentage of people with PMR either have or will develop GCA, and vice versa.
  • Environmental factors: Some research suggests that infections or other environmental triggers might play a role in initiating the condition in genetically susceptible individuals, but no specific trigger has been definitively identified.

Symptoms:

The symptoms of Temporal Arteritis can develop quickly and may vary, but commonly include:

  • Persistent, severe headache: Often localized to the temples, but can be on one or both sides of the head, or in the back of the head. The pain may be throbbing or sharp.
  • Scalp tenderness: Pain or tenderness when touching the scalp, especially over the temporal arteries.
  • Jaw pain (jaw claudication): Pain or cramping in the jaw muscles that worsens with chewing.
  • Vision problems: Sudden, temporary, or permanent loss of vision in one or both eyes (often described as a “curtain coming down”), double vision, or blurred vision. This is a medical emergency.
  • Fatigue: General tiredness and weakness.
  • Fever: Low-grade fever is common.
  • Weight loss: Unexplained weight loss.
  • Loss of appetite.
  • Muscle aches and stiffness: Similar to polymyalgia rheumatica, affecting the shoulders, neck, and hips.

Diagnosis:

Diagnosing Temporal Arteritis is crucial and often involves a combination of:

  • Medical history and physical examination: The doctor will ask about your symptoms, especially headaches, jaw pain, and vision changes. They will also examine your temporal arteries, which may be tender, swollen, or have a reduced pulse.
  • Blood tests:
    • Erythrocyte Sedimentation Rate (ESR): A significantly elevated ESR is a strong indicator of inflammation.
    • C-reactive protein (CRP): Another marker of inflammation that is typically high in GCA.
    • Other blood tests may be done to rule out other conditions.
  • Temporal artery biopsy: This is often considered the gold standard for diagnosis. A small sample of the temporal artery is surgically removed and examined under a microscope for signs of inflammation (giant cells).
  • Imaging tests:
    • Ultrasound: High-resolution ultrasound of the temporal arteries can show signs of inflammation (e.g., “halo sign”).
    • MRI or CT angiography: May be used to visualize larger arteries and assess for inflammation or narrowing.
    • PET scan: Can sometimes identify inflammation in large arteries throughout the body.

Treatment:

Treatment for Temporal Arteritis is a medical emergency aimed at preventing irreversible complications, especially vision loss.

  • High-dose corticosteroids:
    • Oral corticosteroids (e.g., prednisone) are the cornerstone of treatment and are started immediately, often even before a biopsy confirms the diagnosis, especially if vision changes are suspected.
    • The initial dose is high to rapidly control inflammation and protect vision.
    • The dose is gradually tapered over many months to years, as GCA is a chronic condition that requires long-term management.
  • Immunosuppressants:
    • In some cases, other immunosuppressive medications (e.g., methotrexate, tocilizumab) may be added to allow for a lower corticosteroid dose, reduce side effects, or manage refractory disease.
  • Aspirin: Low-dose aspirin may be recommended to help prevent blood clots and reduce the risk of stroke or heart attack.
  • Bone-preserving medications: Calcium, Vitamin D, and bisphosphonates are often prescribed to counteract the bone-thinning side effects of long-term corticosteroid use.
  • Monitoring: Regular follow-up with your doctor is essential to monitor symptoms, inflammation markers (ESR, CRP), and adjust medication dosage. Close monitoring for potential complications is also crucial.

Early diagnosis and prompt treatment with corticosteroids are critical to prevent permanent vision loss and other serious complications of Temporal Arteritis.