Understanding Vasculitis: Causes, Symptoms, Treatment and Prevention

Vasculitis refers to inflammation of a blood vessel, such as an artery or vein. The inflammation can cause the walls of the vessel to thicken and narrow, potentially restricting blood flow to vital organs and tissues. If vasculitis affects the heart, it can lead to serious complications, such as inflammation of the heart muscle (myocarditis), inflammation of the coronary arteries (coronary arteritis) or valvular disease, all of which can result in congestive heart failure.

Recognizing and understanding vasculitis is essential for maintaining optimal cardiovascular health. Timely diagnosis and treatment are needed to control the inflammation, preserve heart function and reduce the risk of severe complications.

What Are the Types of Vasculitis?

Vasculitis may be classified as large, medium or small based on the size of the affected blood vessel. Each type has distinct clinical features and requires specific management strategies. The types of large vessel vasculitis include:

• Giant cell arteritis (GCA) – Inflammation of a large artery, such as a temporal artery, can lead to headaches, jaw pain and vision problems.
• Takayasu arteritis – Inflammation of the aorta or its main branches can cause arm weakness, chest pain or high blood pressure.

The types of medium vessel vasculitis include:

• Polyarteritis nodosa (PAN) – PAN targets medium-sized arteries, potentially causing skin ulcers, muscle pain and abdominal issues.
• Kawasaki disease – A type of vasculitis that primarily affects children, Kawasaki disease causes inflammation in medium-sized arteries throughout the body, which can result in fever, skin rashes and swollen lymph nodes.

The types of small vessel vasculitis include:

• Granulomatosis with polyangiitis (GPA) – Formerly known as Wegener’s granulomatosis, GPA involves inflammation of small blood vessels in the nose, sinuses, lungs and kidneys, which can cause nasal congestion, coughing and kidney issues.
• Microscopic polyangiitis (MPA) – MPA affects small blood vessels, particularly in the kidneys and lungs, which can cause weight loss, skin lesions or kidney dysfunction.
• Eosinophilic granulomatosis with polyangiitis (EGPA) – Previously known as Churg-Strauss syndrome, EGPA involves small vessel inflammation associated with asthma and eosinophilia, which can cause both respiratory and systemic symptoms.

What Causes Vasculitis and What Are the Risk Factors?

Vasculitis occurs when the body’s immune system mistakenly attacks blood vessel cells, leading to inflammation. Known risk factors include:

• Young or advanced age – Kawasaki disease is most common in children younger than five, while giant cell arteritis typically affects adults older than 50.
• Family history – A familial predisposition exists for certain types of vasculitis, such as Kawasaki disease, suggesting a genetic component.
• Autoimmune conditions – Lupus, rheumatoid arthritis and scleroderma are associated with blood vessel inflammation.
• Infection – Chronic infections, particularly hepatitis B or hepatitis C, can trigger the immune response that causes vasculitis.
• Cancer – Lymphoma and breast, colon, kidney, prostate, stomach, thyroid and non-small cell lung cancers have been linked to vasculitis.
• Medications – A reaction to certain antibiotics, anti-thyroid drugs, anti-tumor necrosis factor-a agents and psychoactive agents can lead to blood vessel inflammation.
• Unhealthy lifestyle habits – Tobacco use, especially in men younger than 45, and illegal drug use, such as cocaine, are risk factors for vasculitis.
• Environmental factors – Prolonged exposure to certain harmful substances, such as silica dust, air pollutants and heavy metals, can increase the risk of blood vessel inflammation.

In some cases, the exact cause of vasculitis is unknown.

What Are the Warning Signs and Symptoms of Vasculitis?

Vasculitis can cause a variety of symptoms based on the condition type and organs involved. General warning signs can include fever, fatigue, unexplained weight loss and general aches and pains, which may be accompanied by specific symptoms affecting the:

• Skin – Rashes and discoloration
• Joints – Pain and swelling
• Lungs – Coughing and shortness of breath
• Kidneys – Dark or bloody urine
• Nervous system – Numbness and tingling, especially in the hands and feet

How Is Vasculitis Diagnosed?

Diagnosing vasculitis generally requires a thorough and systematic approach to confirm the condition and assess its type and severity. Typically, the process begins with a physical examination, symptom assessment and review of the patient’s medical history. Next steps may include:

• Laboratory testing – Blood work can detect signs of inflammation, such as elevated levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Additionally, the presence of specific antibodies in the bloodstream, such as anti-neutrophil cytoplasmic antibodies (ANCA), can support the diagnosis of certain types of vasculitis.
• Imaging studies – X-rays, magnetic resonance imaging (MRI) scans, computed tomography (CT) scans and positron emission tomography (PET) scans can help a physician visualize blood vessels and assess organ inflammation, aiding in the diagnosis of vasculitis.
• Biopsy – A definitive diagnosis of vasculitis often requires a biopsy, which involves removing a small sample of tissue from an affected area, such as the skin or a kidney, lung, nerve or temporal artery. The tissue will be microscopically examined by a pathologist, who can identify blood vessel inflammation and confirm the presence of vasculitis.

How Is Vasculitis Treated?

Vasculitis treatment can vary based on the condition’s type, severity and underlying cause, as well as the organs and tissues affected by the blood vessel inflammation. Options may include:

Treatment of Underlying Health Issues

Effective treatment of vasculitis often involves addressing the underlying cause, such as:

• Infection – If vasculitis is triggered by an infection, resolving the infection with antibiotic, antiviral or antifungal medication may help resolve the blood vessel inflammation.
• Autoimmune condition – If vasculitis is associated with an autoimmune disease, such as lupus or rheumatoid arthritis, treatment often focuses on managing the primary condition with a combination of medication and lifestyle modifications.

Medications

Several types of medication can be used to reduce blood vessel inflammation and manage the body’s immune response:

• Corticosteroids – Commonly prescribed to reduce inflammation, drugs such as prednisone may be used as a first-line treatment for vasculitis. Typically, a high dose is prescribed initially and then tapered gradually as symptoms improve.
• Immunosuppressants – If corticosteroids are ineffective or cause severe side effects, an immunosuppressive agent such as methotrexate, azathioprine or cyclophosphamide may be prescribed to prevent the immune system from targeting healthy cells.
• Biologic therapies – Targeted treatments such as rituximab may be used for specific types of vasculitis, such as ANCA-associated vasculitis, to help regulate the immune system. Biologics may cause fewer side effects than some traditional immunosuppressants.

Surgical and Interventional Procedures

In some cases, surgical or interventional treatment may be considered. For instance, surgery may be needed to address complications of vasculitis, such as an aneurysm, blocked artery or damaged organ.

Ongoing Monitoring and Management

Patients with vasculitis often benefit from long-term care and support, which may include:

• Follow-up visits – Regular consultations with a health care provider are essential to check for signs of vasculitis relapse and treatment side effects.
• Medication adjustments – Over time, medications may be adjusted or discontinued based on the patient’s response to therapy.
• Pain management – Nonsteroidal anti-inflammatory drugs (NSAIDs) and pain relievers can be used as needed to manage discomfort.
• Blood pressure control – Medications may be prescribed to reduce blood pressure, which can protect the kidneys and other organs affected by blood vessel inflammation.
• Lifestyle adjustments – Dietary changes, regular physical activity and stress management can support overall health during vasculitis treatment.

What Are the Complications and Long-Term Effects of Vasculitis?

Vasculitis has several potential complications and long-term effects, which can vary depending on the severity of the blood vessel inflammation and the organs and tissues affected. These include:

• Organ damage – Chronic inflammation can damage organs and other structures throughout the body, such as the kidneys, lungs, heart and nervous system, potentially resulting in long-term dysfunction.
• Blood clots – Swelling from inflammation may narrow blood vessels, slowing the flow of blood and encouraging clot formation, which in turn can increase the risk of heart attack, stroke and deep vein thrombosis (DVT).
• Recurrent infections – Some immunosuppressive treatments used to manage vasculitis can weaken the immune system, making the patient more susceptible to infection.
• High blood pressure – Inflammation of the blood vessels in the kidneys (renal vasculitis) can impair their ability to filter blood effectively, leading to fluid retention and increased blood volume, both of which can raise blood pressure.
• Nerve damage – Vasculitis can cause swelling and narrowing in the small blood vessels that supply nerves, reducing blood flow. The resulting lack of oxygen and nutrients can lead to nerve dysfunction or damage.
• Chronic fatigue – Many people with vasculitis experience long-term fatigue from both the condition and its treatment.
• Vision problems – If vasculitis affects blood vessels in the eyes, it can lead to vision impairment or blindness.
• Blood vessel scarring – Ongoing inflammation can cause blood vessels to scar and narrow, reducing blood flow to vital organs and tissues.

How Can Vasculitis Be Prevented?

Vasculitis cannot be completely prevented because many of its triggers cannot be controlled. That said, there are several ways to reduce the risk of developing vasculitis and manage the condition effectively if it does develop. These include:

• Detecting and treating health issues – Prompt detection and treatment of underlying health issues, such as infections and autoimmune conditions, can help prevent vasculitis. Regular medical check-ups and monitoring are essential, especially if there is a family history of autoimmune disease.
• Avoiding infections – Certain infections, such as those caused by bacteria or viruses, can trigger vasculitis in some people. Practicing proper hygiene and receiving appropriate vaccinations can help reduce the risk. It is also important to promptly treat any infections that do develop.
• Managing autoimmune conditions – If an autoimmune disease is diagnosed, a health care provider can suggest an appropriate management strategy to help reduce the likelihood of developing vasculitis.
• Adopting a healthy lifestyle – Consuming a balanced diet, engaging in regular physical activity and managing stress can help support a healthy immune system. Avoiding tobacco use and limiting alcohol consumption can further reduce the risk of developing vasculitis.
• Taking immunosuppressants as directed – In some cases, a physician may prescribe an immunosuppressive medication to manage immune system activity and help prevent inflammation that could trigger vasculitis.
• Minimizing exposure to known environmental risk factors – Vasculitis has been linked to certain harmful substances, so it is important to take steps to avoid exposure in the workplace and elsewhere.

Living With Vasculitis

In general, living with vasculitis requires careful management, adaptability and a strong support system. While each patient’s circumstances are unique, some common experiences and challenges faced by those with vasculitis include:

• Physical symptoms – Vasculitis can cause fatigue, muscle and joint pain, fever, weight loss and skin rashes. These symptoms may be chronic or intermittent, making it difficult to maintain a routine.
• Frequent medical appointments – Ongoing treatment and monitoring are essential, often requiring frequent physician visits and lab tests. For some people, this can become time-consuming and stressful.
• Impact on daily life – Depending on the severity of its symptoms, vasculitis can interfere with work, family responsibilities, social activities and daily tasks. Chronic pain, fatigue and other symptoms can make it difficult to stay active and participate in certain events, which could lead to isolation or a sense of loss.
• Medication side effects – Some people with vasculitis take immunosuppressive medications, which can cause side effects such as increased susceptibility to infection, weight gain, osteoporosis and mood swings. Managing these side effects can be challenging and may require treatment adjustments.
• Mental and emotional impact – The unpredictable nature of vasculitis can lead to anxiety, stress or depression. Additionally, its physical symptoms and impact on daily life can cause emotional strain, making it important to seek mental health support if needed.
• Flare-ups – People with vasculitis often experience sporadic episodes of worsening symptoms that may require treatment adjustments or hospitalization, potentially leading to disruption and frustration.
• Long-term health considerations – If vasculitis damages vital organs, such as the kidneys, heart or lungs, managing the resulting issues can become a lifelong challenge. Ongoing monitoring and interventions may be needed to prevent further complications.
• Lifestyle adaptations – Many people with vasculitis adapt by making positive lifestyle changes, such as maintaining a healthy diet, getting regular exercise and reducing stress. These changes may alleviate symptoms and improve overall well-being.

Frequently Asked Questions (FAQs) About Vasculitis

The following FAQs and corresponding answers provide additional information about vasculitis:

Is vasculitis contagious?

No, vasculitis is not transmissible. Rather, it is an inflammatory condition that occurs when the body’s immune system mistakenly attacks blood vessels. It cannot be spread from one person to another.

Does vasculitis only affect older adults?

No, vasculitis affects individuals of all ages, including children. While some types of vasculitis, such as giant cell arteritis, are more common in older adults, other forms, like Kawasaki disease, primarily affect children.

Is vasculitis always a lifelong condition?

Not necessarily. Some types of vasculitis, particularly those triggered by infection or medication, may resolve after the trigger is removed or treated. However, chronic forms of vasculitis may require long-term management to prevent flare-ups and complications.

Does vasculitis always cause severe symptoms?

No, the severity of vasculitis symptoms can vary widely. Some people experience mild symptoms, such as fatigue and rash, while others may have serious complications affecting major organs. Early diagnosis and treatment can help minimize the severity in many cases.

Can vasculitis be cured?

There is no universal cure for vasculitis, but many types can be effectively managed with treatment. Some people achieve long-term remission, which means they have no symptoms or disease activity for an extended period.

Is vasculitis the same as arthritis?

No, although both vasculitis and arthritis can cause joint pain, the conditions are distinct. Vasculitis involves blood vessel inflammation, while arthritis involves joint inflammation. However, vasculitis is a complication of rheumatoid arthritis, an autoimmune disorder.

Does everyone with vasculitis require immunosuppressive drugs?

Not everyone. Vasculitis treatment can vary widely depending on the type and severity of the condition. A mild case may only require anti-inflammatory medication, while a severe case affecting vital organs may need immunosuppressive therapy.

Can lifestyle changes cure vasculitis?

Healthy lifestyle practices, such as consuming a nutritious diet, exercising regularly, managing stress and avoiding tobacco use, can support overall health and complement medical treatment, but these strategies alone cannot cure vasculitis. Medical management of the condition is essential.

Is vasculitis caused by poor lifestyle choices?

No, vasculitis is not caused by poor lifestyle choices. Instead, it is often linked to autoimmune or genetic factors, although infections and medications can trigger some forms. Healthy habits can help manage symptoms but cannot prevent the condition outright.

Does vasculitis always lead to organ failure?

Not always. Untreated or severe vasculitis can potentially cause organ damage, but many cases are caught early and managed effectively, avoiding the development of serious complications. Regular monitoring and adherence to treatment are crucial for minimizing risks.

Benefit From World-Class Care at TGH

The multidisciplinary team in Tampa General Hospital’s Heart and Vascular Institute provides comprehensive, individualized care for patients with heart conditions. We are proud to offer the latest treatment options for vasculitis, including immunosuppressants and biologic therapies. In acknowledgment of our commitment to providing outstanding care, U.S. News & World Report has recognized TGH among the nation’s best hospitals for heart and vascular care for 2024-25.

As a leading academic health system, TGH regularly collaborates with the University of South Florida (USF) Health Morsani College of Medicine on cutting-edge research and groundbreaking clinical trials. Additionally, we handle some of the nation’s most complex cardiac cases while prioritizing patient comfort and safety.

For more information, contact us at (813) 844-3900 and request an appointment with a specialist on our team.