Giant Cell Arteritis

Giant Cell Arteritis: Symptoms, Causes, Types, Diagnosis, and Treatments

Giant Cell Arteritis (GCA), also known as temporal arteritis or cranial arteritis, is a serious and potentially sight-threatening condition characterized by inflammation of the lining of the arteries. It primarily affects the large and medium-sized arteries of the head, particularly the temporal arteries, which run along the temples. Left untreated, GCA can lead to irreversible vision loss or even stroke. Early detection and prompt treatment are crucial for preventing complications and improving outcomes.

This comprehensive guide explores the symptoms, causes, types, diagnostic approaches, and treatment options for Giant Cell Arteritis, providing essential information for patients, caregivers, and healthcare professionals.

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Understanding Giant Cell Arteritis

Giant Cell Arteritis is an autoimmune vasculitis that causes inflammation of blood vessels. The disease is termed “giant cell” because biopsy specimens from affected arteries often show large cells (giant cells) formed by the fusion of macrophages — a type of immune cell. GCA most often affects people over the age of 50 and is more common in women than men. It is closely linked to another inflammatory disorder called polymyalgia rheumatica (PMR), which causes muscle pain and stiffness, particularly in the shoulders and hips.

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Symptoms of Giant Cell Arteritis

GCA symptoms may vary from person to person and can develop gradually or come on suddenly. Early symptoms may resemble those of the flu, making diagnosis challenging in the initial stages. Common symptoms include:

1. Headache

• The hallmark symptom of GCA.
• Usually a new onset, localized to the temples.
• May be severe, persistent, and resistant to usual painkillers.

2. Scalp Tenderness

• Pain or discomfort when combing hair or touching the scalp.
• Indicative of inflamed arteries under the scalp.

3. Jaw Claudication

• Pain in the jaw muscles during chewing or talking.
• Occurs due to reduced blood flow to the jaw muscles.

4. Vision Problems

• Blurred or double vision.
• Sudden, painless vision loss in one eye (a medical emergency).
• Can lead to permanent blindness if untreated.

5. Fatigue and Malaise

• Generalized tiredness, weakness, and low energy.
• May be accompanied by weight loss and low-grade fever.

6. Polymyalgia Rheumatica (PMR) Symptoms

• Stiffness and aching in the shoulders, neck, and hips.
• Worse in the morning or after periods of inactivity.

7. Fever and Night Sweats

• Low-grade fever, often mistaken for an infection.
• Can occur with or without other systemic symptoms.

8. Tender Temporal Arteries

• The arteries on the side of the head may feel thickened, tender, or pulseless.

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Causes and Risk Factors of Giant Cell Arteritis

The exact cause of GCA is not fully understood, but it is believed to be an autoimmune condition, where the body’s immune system mistakenly attacks its own blood vessels. Various genetic and environmental factors may contribute to the development of GCA.

1. Age

• Almost exclusively affects individuals over 50.
• Risk increases with advancing age.

2. Gender

• Women are about two to three times more likely to develop GCA than men.

3. Ethnicity

• Most common among people of Northern European descent, especially those of Scandinavian origin.

4. Genetics

• Family history may increase the risk.
• Certain genetic markers, such as HLA-DR4, are associated with a higher incidence.

5. Infections

• Some studies suggest a link between viral infections and the onset of GCA.
• The exact pathogens and mechanisms remain under investigation.

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Types of Giant Cell Arteritis

GCA primarily affects the arteries of the head and neck, but in some cases, other arteries may be involved. It can be classified based on the arteries affected and the associated conditions.

1. Cranial GCA

• Affects the temporal arteries and other branches of the external carotid artery.
• Most common type.
• Presents with headaches, scalp tenderness, and vision disturbances.

2. Large-Vessel GCA

• Involves the aorta and its major branches (e.g., subclavian, carotid).
• May cause arm claudication, chest pain, or asymmetrical pulses.
• Can be asymptomatic or present subtly compared to cranial GCA.

3. GCA with Polymyalgia Rheumatica

• Co-occurs with PMR in about 40-60% of cases.
• Patients may initially present with PMR symptoms before GCA signs appear.

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Diagnosis of Giant Cell Arteritis

Prompt diagnosis of GCA is crucial due to the risk of sudden and irreversible vision loss. A thorough clinical evaluation combined with specific tests helps confirm the diagnosis.

1. Medical History and Physical Examination

• Evaluation of symptoms and risk factors.
• Palpation of temporal arteries for tenderness or absent pulse.

2. Blood Tests

• Erythrocyte Sedimentation Rate (ESR): Usually elevated (>50 mm/hr).
• C-Reactive Protein (CRP): Elevated; often more sensitive than ESR.
• Complete Blood Count (CBC): May show anemia and elevated platelets.

3. Temporal Artery Biopsy

• Gold standard for diagnosis.
• A small section of the temporal artery is surgically removed and examined under a microscope.
• Reveals inflammation, giant cells, and damage to vessel walls.

4. Imaging Studies

• Ultrasound: Can detect “halo sign” (vessel wall swelling).
• MRI and CT Angiography: Useful for detecting large-vessel involvement.
• PET Scan: Highlights inflammation in large arteries.

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Differential Diagnosis

Several conditions may mimic GCA, making differential diagnosis essential:

• Polymyalgia Rheumatica without GCA
• Migraine or tension headaches
• Infectious causes like meningitis
• Other types of vasculitis (e.g., Takayasu arteritis)
• Sinusitis or temporal bone disorders

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Treatment Options for Giant Cell Arteritis

Early and aggressive treatment is essential to prevent vision loss and other complications. The mainstay of treatment is corticosteroid therapy.

1. Corticosteroids

• Prednisone is typically started immediately upon suspicion of GCA.
• High doses (40-60 mg/day) are used initially.
• Symptoms often improve within days.
• Gradual tapering is done over months based on symptoms and inflammatory markers.
• May be continued for 1-2 years.

2. Intravenous Methylprednisolone

• Used in cases with acute visual loss.
• Followed by high-dose oral corticosteroids.

3. Steroid-Sparing Agents

• Used to reduce long-term corticosteroid exposure and side effects.
• Tocilizumab (Actemra): IL-6 receptor blocker approved for GCA.
• Methotrexate: Sometimes used as adjunctive therapy.
• Azathioprine or Leflunomide: Used in select cases.

4. Aspirin

• Low-dose aspirin may reduce the risk of stroke and vision loss by preventing blood clots.

5. Calcium and Vitamin D

• Prescribed to prevent steroid-induced osteoporosis.
• Bone density monitoring is recommended.

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Lifestyle and Supportive Care

Living with GCA requires managing symptoms, minimizing treatment side effects, and staying vigilant for relapses.

1. Diet and Nutrition

• Anti-inflammatory diet (rich in fruits, vegetables, omega-3 fatty acids).
• Limit salt and sugar to counteract steroid side effects.

2. Exercise

• Regular low-impact exercise helps maintain bone health and muscle strength.

3. Monitoring

• Regular follow-ups to monitor disease activity and adjust medications.
• Vision monitoring and ophthalmology assessments.

4. Mental Health

• Chronic illness and long-term steroid use can affect mood and sleep.
• Counseling, support groups, and therapy can help.

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Prognosis and Long-Term Outlook

With prompt diagnosis and appropriate treatment, the prognosis for GCA is generally favorable. Most patients respond well to corticosteroids, and vision loss can be prevented if treated early. However, relapses are common and may require long-term immunosuppressive therapy.

Complications to Watch For

• Vision Loss: May occur suddenly and permanently.
• Aortic Aneurysm or Dissection: Particularly in patients with large-vessel involvement.
• Relapses: Even after remission, symptoms can return.

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Conclusion

Giant Cell Arteritis is a serious medical condition that demands immediate attention. Characterized by inflammation of the arteries, especially in the head and neck, it can lead to devastating consequences if not promptly diagnosed and treated. Recognizing the symptoms—such as new headaches, jaw claudication, and vision changes—can be life-saving.

Thanks to advancements in diagnostic tools and the availability of steroid-sparing medications like tocilizumab, the management of GCA has significantly improved. A collaborative approach involving rheumatologists, ophthalmologists, and primary care providers is essential for optimal patient care.

Frequently Asked Questions About Giant Cell Arteritis

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