Large vessel vasculitis is a group of autoimmune diseases that cause inflammation of the large arteries in the body, such as the aorta and its major branches.
What is Large Vessel Vasculitis?
Large vessel vasculitis is a category of vasculitis (blood vessel inflammation) affecting large arteries. These diseases can disrupt blood flow, causing tissue damage and various symptoms depending on which organs are affected. Two primary types of large vessel vasculitis are Giant Cell Arteritis (GCA) and Takayasu’s Arteritis (TA).
Types of Large Vessel Vasculitis
- Giant Cell Arteritis (GCA) – Also known as temporal arteritis, this condition primarily affects the arteries of the head, particularly the temporal arteries. It commonly occurs in people over 50.
- Takayasu’s Arteritis (TA) – Affects the aorta and its main branches. It primarily occurs in young women under the age of 40.
Symptoms of Large Vessel Vasculitis
The symptoms of large vessel vasculitis can develop gradually and may vary in severity. Common symptoms include:
General Symptoms
- Fever – Persistent or recurrent high temperature.
- Fatigue – Severe tiredness and lack of energy.
- Weight Loss – Unintended loss of weight.
- Muscle and Joint Pain – Generalized pain and discomfort.
Organ-Specific Symptoms
- Headache – Severe, persistent headaches, often around the temples.
- Jaw Claudication – Pain in the jaw muscles while chewing.
- Vision Problems – Blurred or double vision, sudden vision loss.
- Arm or Leg Claudication – Pain in the limbs during physical activity due to reduced blood flow.
- Chest Pain – Discomfort or pain in the chest area.
Symptoms Specific to Giant Cell Arteritis
- Scalp Tenderness – Sensitivity or pain in the scalp, especially over the temples.
- Polymyalgia Rheumatica – Aching and stiffness in the neck, shoulders, and hips.
Symptoms Specific to Takayasu's Arteritis
- Diminished Pulses – Weak or absent pulses in the arms or legs.
- High Blood Pressure – Particularly in the arms.
- Neurological Symptoms – Dizziness, fainting, or stroke-like symptoms.
Causes and Risk Factors
The exact cause of large vessel vasculitis is not fully understood, but it is believed to result from a combination of genetic, environmental, and immune system factors:
- Genetic Predisposition – Certain genetic markers may increase the risk of developing these diseases.
- Autoimmunity – The immune system mistakenly attacks healthy blood vessels, causing inflammation.
- Infections – Certain viral or bacterial infections may trigger the immune system to attack blood vessels.
- Age – GCA commonly affects people over 50, while TA primarily affects young women under 40.
- Gender – Women are more commonly affected by large vessel vasculitis than men.
Diagnosis for Large Vessel Vasculitis
Diagnosing large vessel vasculitis involves a comprehensive evaluation by a healthcare professional, including:
- Symptom Inquiry – Detailed questions about symptoms, their onset, duration, and severity.
- Past Medical History – Inquiry into any history of infections, autoimmune diseases, or other relevant conditions.
- Vascular Assessment – Checking for pulses, blood pressure differences, and signs of vascular insufficiency.
- Temporal Artery Exam – In GCA, palpating the temporal arteries for tenderness and thickening.
- Blood Tests – To check for markers of inflammation (e.g., ESR, CRP) and specific autoantibodies.
- Ultrasound – To visualise inflammation in the temporal arteries or other affected vessels.
- MRI or CT Angiography – To assess large vessels for narrowing, thickening, or aneurysms.
- PET Scan – To detect inflammation in blood vessels.
- Temporal Artery Biopsy – In GCA, a sample of the temporal artery may be taken to confirm the diagnosis.
- Other Biopsies – As needed based on the location of affected vessels.
Large Vessel Vasculitis Treatment Options
The treatment of large vessel vasculitis aims to reduce inflammation, prevent organ damage, and manage symptoms. Treatment options include:
Medications
- Corticosteroids – To reduce inflammation and suppress the immune system.
- Immunosuppressants – Drugs such as methotrexate, azathioprine, or cyclophosphamide to control the immune response.
- Biologic Agents – Targeted therapies such as tocilizumab to reduce inflammation, particularly in GCA.
Supportive Care
- Pain Management – Analgesics to manage pain and discomfort.
- Antihypertensives – Medications to control high blood pressure, particularly in TA.
Lifestyle Modifications
- Healthy Diet – Eating a balanced diet supports overall health and manages symptoms.
- Regular Exercise – Engaging in low-impact physical activities to maintain general fitness.
Regular Medical Follow-Up
- Monitor Progress – Regular visits to your healthcare provider to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns.
Managing and Living with Large Vessel Vasculitis
Living with large vessel vasculitis involves a combination of medical treatment, lifestyle changes, and self-care strategies. Here are some tips:
- Take medications as your healthcare provider prescribes to control inflammation and prevent disease progression.
- To maintain physical fitness and improve symptoms, engage in regular, low-impact exercises such as walking, swimming, or yoga.
- Eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health.
- Practice stress-reduction techniques such as meditation, deep breathing exercises, and mindfulness to help manage symptoms.
- Stay current with vaccinations and practice good hygiene to reduce the risk of infections, which can trigger flare-ups.
- Visit your healthcare provider regularly to monitor disease activity, adjust treatment as needed, and address any new symptoms or concerns.
Large vessel vasculitis is a complex and serious condition that requires comprehensive management. While there is currently no cure, with early diagnosis and appropriate treatment, many individuals can manage their symptoms and maintain a good quality of life. If you suspect you have large vessel vasculitis or are experiencing symptoms suggestive of the condition, it’s essential to seek medical evaluation and treatment promptly. By working closely with your healthcare team, adhering to treatment plans, and making healthy lifestyle choices, you can effectively manage large vessel vasculitis and improve your quality of life.
