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Understanding Giant Cell Arteritis: Symptoms, causes & treatments

Team AckoJun 23, 2024

It's crucial to consult a healthcare professional if experiencing symptoms like throbbing headaches, jaw pain, or vision changes, as they could indicate Giant Cell Arteritis (GCA). This autoimmune disease affects blood vessels in the head, neck, and upper arms, causing severe and potentially debilitating symptoms. Early diagnosis and treatment are vital to managing GCA and preventing complications. Seeking medical attention promptly can help alleviate symptoms and improve the quality of life for those affected by this condition. In this article, you will know the symptoms, causes, diagnosis, and treatment options for GCA and how you can manage it if you’re living with it.

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Contents

What is Giant Cell Arteritis?

Giant Cell Arteritis is a type of vasculitis that causes inflammation of blood vessels, specifically of the arteries in the head, neck, and upper limbs. It is also called temporal arteritis. This inflammation can cause damage to the artery walls leading to narrowing, constriction, and hardening of the artery. If left untreated, the condition can lead to permanent and severe vision loss.

Who can get giant cell arteritis?

Giant cell arteritis (GCA) is a rare condition more prevalent in women and individuals of North European descent, often associated with polymyalgia rheumatica (PMR). PMR presents with muscle pain and stiffness, particularly in the shoulders, neck, hips, and thighs, with notable morning stiffness. GCA entails inflammation of head and neck arteries, with ongoing research into its exact causes.

Timely diagnosis and treatment are crucial to prevent severe complications such as vision loss and stroke. Recognising symptoms promptly and seeking medical attention is imperative for optimal management and reducing the risk of adverse outcomes. Early intervention can significantly improve prognosis and quality of life for those affected by GCA and PMR.

Signs of Giant Cell Arteritis

Here is a list of common symptoms of GCA.

Headache, often concentrated on one side of the head

Jaw pain (particularly when chewing)

Fatigue

Fever

Vision changes, including double vision, blurred vision, or temporary vision loss

Scalp tenderness

Pain in the neck, shoulders, arms, or upper chest

Loss of appetite

Unusual weight loss

Minor strokes

Temporal artery tenderness

Unusual scalp growths

Muscle aches or pains

Numbness or tingling in the face or limb

Sensitivity to light

Causes of Giant Cell Arteritis

It is primarily seen in people over the age of 50 and is more common in women than men. The exact cause of GCA is unknown, but it is believed that genetic and environmental factors both play a role.

Genetic factors: Genetic factors are thought to be an important contributor to GCA. Several genetic traits have been linked to GCA, including certain HLA alleles, or genetic markers, and a gene known as CDKN2A, which is associated with an increased risk of this condition.

Environmental factors: Environmental factors, such as smoking, air pollution, and exposure to certain viruses or bacteria, have been linked to an increased risk of GCA. Also, people with autoimmune conditions, such as rheumatoid arthritis, may be more likely to develop GCA.

Immunological factors: In some cases, the immune system might play a role in the development of GCA. Specifically, certain chemicals released by the immune system, such as cytokines, may stimulate the inflammation that causes this condition. There is also evidence that the immune system might be reacting to a foreign substance, such as bacteria or a virus, in the body. This could explain why some people are more likely to develop such a condition than others.

Giant cell arteritis (GCA) is often associated with other rheumatic conditions, notably polymyalgia rheumatica (PMR) and large vessel vasculitis (LVV). PMR is characterised by pain and stiffness, predominantly affecting the shoulders, neck, hips, and thigh muscles. These symptoms are typically worse in the morning, making daily activities like dressing, reaching, or climbing stairs challenging. Individuals with PMR may also experience general malaise, slight fever, weight loss, and overwhelming fatigue.

CGA and PMR: What’s the connection?

Approximately half of those diagnosed with GCA may also exhibit symptoms of PMR, suggesting a potential connection between the two conditions. Moreover, individuals previously treated for PMR might develop GCA later in life, underscoring the need for continued monitoring and awareness of overlapping symptoms.

CGA and LVV

In addition to PMR, GCA can be associated with large vessel vasculitis (LVV), characterised by inflammation of blood vessels beyond the head and neck. While GCA primarily affects arteries in the head and neck, LVV may involve inflammation of the aorta and vessels supplying the head, neck, and arms. Symptoms of LVV can include weight loss, anaemia, night sweats, pains in the arms, legs, and spine, as well as continuous inflammation.

The prevalence of LVV is not definitively established, but individuals experiencing recurrent flares despite steroid treatment may prompt further investigations by their healthcare providers. Diagnostic tests and imaging studies may be recommended to assess the extent of vascular inflammation and tailor treatment accordingly.

Managing LVV

Management of LVV typically involves a multidisciplinary approach aimed at suppressing the immune system and controlling inflammation. Besides steroids, additional medications such as methotrexate, leflunomide, and newer biological drugs like tocilizumab may be utilised to achieve disease control and alleviate symptoms.

Close monitoring and regular follow-up appointments are essential to evaluate treatment efficacy, adjust medications as needed, and monitor for potential side effects.

Overall, recognising the connection between GCA, PMR, and LVV is crucial for timely diagnosis and effective management. Clinicians should maintain a high level of alertness for these conditions, particularly in older adults presenting with characteristic symptoms such as muscle pain, stiffness, constitutional symptoms, and signs of vascular inflammation. Collaborative efforts between patients and healthcare providers are paramount in optimising outcomes and reducing the risk of complications associated with these rheumatic conditions.

Risk factors of Giant Cell Arteritis

Some of the common risk factors associated with this condition are as follows.

Age: GCA typically begins after age 50, and the risk increases with age.

Gender: It is more common in women than in men.

Ethnicity: People of Scandinavian descent, particularly Swedish citizens, are vulnerable to developing this condition.

Cigarette smoking: Cigarette smoking has been associated with an increased risk of GCA

Other autoimmune diseases: People who have had or currently have other autoimmune diseases, such as rheumatoid arthritis, may be at greater risk of developing such an issue.

Family history: Having a parent or sibling with such a condition increases the odds.

Painful jaw when chewing: Jaw pain or tenderness when chewing can be an early symptom.

Complications of GCA

Giant Cell Arteritis can cause a number of the following serious complications.

Vision loss: The most feared complication is permanent loss of vision. This condition is called ocular ischemic syndrome and is caused by inflammation of the arteries supplying the eye, leading to the insufficient blood supply. This can result in blindness, double vision, and other visual disturbances.

Stroke: The inflammation caused by GCA can also lead to narrowing and blockage of the carotid and vertebral arteries. This can result in a stroke.

Aneurysm: Aneurysms can form in the aorta due to inflammation and weakening of the artery wall.

Intracranial hypertension: Increased pressure within the skull and may result in headaches, confusion, and disorientation.

Myocardial infarction: The inflammation caused can block the blood supply to the heart, resulting in a heart attack.

Peripheral neuropathy: Damage to the nerves of the arms and legs, resulting in numbness, tingling, and pain.

Renal failure: Inflammation of the kidneys and lead to kidney failure.

Diagnosis of Giant Cell Arteritis

Diagnosing Giant Cell Arteritis is typically done through a combination of patient history, physical examination, laboratory tests, and imaging tests.

1. Physical examination

The doctor will take a physical look at the temporal artery and feel it for tenderness, hardness and enlargement.

2. Laboratory tests

Blood tests are done to look for evidence of inflammation and raised levels of C-reactive protein (CRP), sedimentation rate, and other inflammatory markers like interleukin-6.

3. Imaging tests

Using ultrasound imaging, a doctor can look for swelling, areas of closure, and reduced blood flow in the temporal artery. Magnetic resonance imaging (MRI) may be used to look for temporal arteritis involvement in other areas, such as the brain and scalp.

4. Other tests

Depending on the situation and severity, a doctor may also order a temporal artery biopsy to examine for the presence of giant cells, which is a definitive diagnostic test for temporal arteritis.

Different treatment options for Giant Cell Arteritis

The most common treatment option for Giant Cell Arteritis (GCA) is glucocorticoid (steroid) therapy. This type of treatment involves taking a series of daily doses, usually prednisone, for a period of 6 to 12 months. As the doses are slowly reduced, symptoms tend to improve. Pain medications such as ibuprofen and naproxen may be prescribed to reduce inflammation and ease the pain.

In some cases, biological therapies that target certain proteins or cells involved in the autoimmune response may be used. These medications include tocilizumab (Actemra), canakinumab (Ilaris), abatacept (Orencia), rituximab (Rituxan), and anakinra (Kineret).

If medication fails to control GCA, surgical interventions may be an option. These include temporal artery biopsy, where a sample of the artery is taken and examined under a microscope.

In some cases, doctors may suggest lifestyle modifications to help reduce symptoms. These may include stress management techniques, regular exercise, and eating a balanced diet.

How to live with Giant Cell Arteritis

To live with this medical issue, there are several things that can be done. First, it is important to make sure you get plenty of rest and stay hydrated. Avoiding alcohol and caffeine can also help. Eating a healthy, balanced diet is essential to maintain good health while living with GCA.

It is also crucial to talk to your doctor about medications used to treat GCA. In some cases, corticosteroids may be needed to reduce inflammation. These medications can cause serious side effects, so it is important to take them as directed.

In addition to medications, lifestyle changes may be needed to help manage your GCA. Physical activity, stress management, and maintaining a healthy weight can help decrease the risk of complications.

It is vital to follow up with your doctor regularly. Because GCA is an autoimmune disorder, it is imperative to monitor any changes in your symptoms and get regular checkups to make sure the disease is being managed properly.

Prevention of Giant Cell Arteritis

GCA is a systemic large vessel vasculitis, which affects the aorta and its main branches. It is a serious disease and may have potentially devastating consequences if not treated properly. The mainstay of prevention lies in early diagnosis and prompt treatment.

The primary goal in the prevention of this condition is to reduce the risk of permanent visual loss. Visual symptoms are the most common initial symptom of GCA, and time is of the essence for diagnosis and treatment in order to avoid irreparable damage. To mitigate the risk of visual loss, it is important to be acutely aware of the risk factors associated with this condition and to seek prompt medical evaluation at the onset of any headache suggestive of GCA.

Elderly patients, particularly those aged over 65, are at an increased risk. Prednisone is the foundation of treatment for GCA, and early initiation of therapy should be considered for patients with risk factors or suggestive symptoms to reduce the risk of irreversible vision loss.

In addition, patients should be screened or tested for other underlying diseases that can increase the risk of developing this illness such as polymyalgia rheumatica or temporal arteritis. This includes a complete blood count to check for anaemia or infection, erythrocyte sedimentation rate to look for inflammatory activity and autoantibodies, and inflammatory markers to look for inflammatory activity. If any of the tests are positive, further investigations, such as imaging and biopsy, should be carried out to confirm the diagnosis.

Giant Cell Arteritis is a serious disease, so it is important to be aware of the risk factors and to seek prompt medical evaluation for any headaches or visual symptoms. Early initiation of treatment with prednisone is essential to reduce the risk of permanent vision loss.

Clinical Studies and Trials

Medications such as methotrexate and azathioprine, targeting the immune system, may be considered for giant cell arteritis treatment when steroids prove ineffective or intolerable. However, their efficacy is constrained by limited evidence from small-scale studies. Adalimumab and infliximab, when combined with steroids, do not appear to enhance remission rates significantly.

Abatacept exhibits promise based on preliminary research but needs further validation. Ongoing clinical trials are investigating mavrilimumab, sarilumab, and JAK inhibitors like upadacitinib. Giant cell arteritis can induce arterial narrowing and heighten blockage risk; thus, low-dose aspirin might offer potential benefits in mitigating these effects.

Outlook of Giant Cell Arteritis

GCA has an unpredictable course and can cause severe, life-threatening complications if not treated promptly and aggressively. As with many chronic illnesses, it is important for individuals to have an understanding of their diagnosis and embark on treatment as soon as possible.

With early diagnosis and aggressive treatment, most individuals can expect a good prognosis and successful long-term outcomes. Treatment usually comprises a combination of high doses of steroids and other immunosuppressive medications.

It is vital to follow up regularly with your doctor and maintain regular monitoring for disease recurrences, since Giant Cell Arteritis has a high likelihood of recurrence. Also, it is advised to maintain a healthy lifestyle with a balanced diet, regular exercise, and stress management.

Frequently asked questions

Here are some common queries with answers related to Giant Cell Arteritis.

Is Giant Cell Arteritis curable?

Yes. With prompt treatment, this condition is usually curable. Treatment typically includes high-dose steroids, which can reduce inflammation in the arteries. If the disease is recognized and treated early, it can prevent serious lasting damage such as vision loss.

Can you live a long life with Giant Cell Arteritis?

Yes, many people with Giant Cell Arteritis are able to lead long, productive lives. The disease can be managed and the inflammation in the arteries can be reduced if the condition is diagnosed and treated on time. When left untreated, the artery inflammation can cause permanent vision loss or damage to the artery walls leading to stroke, heart attack, or aneurysm.

What are the long-term effects of Giant Cell Arteritis?

Some of the long-term effects of Giant Cell Arteritis may include vision loss, stroke, heart attack, and aneurysms. Damage to the artery walls from inflammation can lead to these serious conditions. Timely treatment is essential to minimise long-term effects.

What are the risk factors for Giant Cell Arteritis?

The risk factors for Giant Cell Arteritis include age, gender, family history and certain autoimmune diseases. The condition is more common in women over the age of 50 and those with a family history of the condition. It is also more common in those with certain autoimmune conditions such as rheumatoid arthritis or lupus.

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