Vasculitis: What You Need to Know

Vasculitis refers to a group of disorders characterized by inflammation of blood vessels. Many types are autoimmune, though some are triggered by infections, medications, or other conditions. While some types are extremely uncommon, overall prevalence varies depending on the specific subtype. In this article, you will learn about its causes, symptoms, diagnosis, and treatment options. Also, we will answer some of the most common questions about this condition.  

What Is Vasculitis?

Synonyms: Angiitis and arteritis

Vasculitis is not a single disease. It is a group of disorders that involve swelling and irritation of blood vessels. When the blood vessels are inflamed and narrowed, they cannot supply enough blood to the organs and tissues. As a result, these organs and tissues can become damaged. 

Vasculitis can affect the following types of blood vessels in the body:

• Arteries (Arteries deliver oxygen-rich blood to different organs and tissues)
• Veins (Veins take oxygen-poor blood to the heart)
• Capillaries (Tiny vessels that carry oxygen and nutrients to cells in the body)

While many types of vasculitis exist, most are rare. Depending on the type of blood vessel affected, this condition may affect one or several organs. 

What Causes Vasculitis?

Researchers have yet to determine the exact cause of vasculitis. Your genes might be responsible for some types, while others might result from your immune system mistakenly attacking the blood vessels. 

Several factors may be responsible for such immune reactions:

• Infections (Hepatitis B and hepatitis C)
• Blood cancers
• Immune disorders like rheumatoid arthritis, lupus, and scleroderma
• Reactions to certain drugs, including quinolones, clarithromycin, furosemide, NSAIDs, and TNF-α inhibitors

Risk Factors

Anyone can get this condition. However, some factors may increase the risk, such as:

• Age: Giant cell arteritis typically affects people older than 50. Kawasaki disease is most common in children below 5 years old.
• Lifestyle factors: Use of tobacco and cocaine may increase the risk of certain types of vasculitis, especially in men younger than 45. 
• Infections and autoimmune conditions: Certain infections and autoimmune disorders can trigger your immune system to attack healthy blood vessels. 

What Are the Symptoms of Vasculitis?

Symptoms common to most types of vasculitis are:

• Fever
• Headache
• Tiredness
• Weight loss
• Aches and pains

Symptoms specific to the part of the body affected can include:

Digestive system

• Pain after eating
• Ulcers
• Blood in stool 

Ear

• Dizziness
• Ringing in the ears
• Sudden hearing loss

Eyes

• Itching, burning, and redness in the eyes 
• Double vision
• Temporary or ongoing blindness

Hands or feet

• Numbness or weakness in hands or feet
• Swollen palms or soles 

Lungs

• Shortness of breath 
• Blood in cough 

Skin

• Red spots due to bleeding under the skin 
• Open sores or lumps 

How Is Vasculitis Diagnosed?

Diagnosis involves a medical history and physical exam. 

Your healthcare provider may order tests to diagnose vasculitis or exclude other conditions with overlapping symptoms. Tests can include:

• Blood tests: They help detect inflammatory markers and certain antibodies (proteins) in your body. 
• Imaging tests: Tests like X-rays, ultrasounds, CT scans, MRIs, and PET scans can help determine the location of the affected blood vessels and organs. 
• Angiography: This test helps your healthcare provider observe early inflammatory changes in your blood vessels. 
• Biopsy: During a biopsy, a healthcare professional removes a small piece of tissue from the affected area. The tissue is further tested in a lab to look for signs of vasculitis. 

What Are the Treatments for Vasculitis?

No cure for vasculitis is currently available. However, appropriate treatments can help you enter symptom-free periods (remission). Remission can last for years or months. 

Treatments help manage your symptoms and prevent damage to your blood vessels and organs. They can include:

Corticosteroids

Prednisone, a corticosteroid, is the most commonly used medication to reduce inflammation. Corticosteroids are typically used during active disease to achieve remission. 

Corticosteroids, especially when used for prolonged periods, can cause side effects, such as:

• Weight gain 
• Elevated blood sugar
• Weakened bones 

Immunosuppressants

These medications help prevent your immune system from attacking the blood vessels. They are used to keep you in remission for as long as possible. Examples include azathioprine, methotrexate, and mycophenolate.

Other medications that may be used based on the type of vasculitis include:

• Tocilizumab (Actemra)
• Rituximab (Rituxan)
• Colchicine (Colcrys)

Surgery 

Surgery may be an option if you have:

• Aneurysms (balloonlike bulges in the wall of a blood vessel)
• Blocked arteries 

Can You Use IVIG for Vasculitis?

Your healthcare provider may prescribe IVIG for vasculitis, but only in specific cases. 

Medications such as rituximab, methotrexate, and azathioprine can lead to favorable outcomes for most patients. However,  IVIG may be an option when a patient cannot take an immunosuppressant because they already have a compromised immune system. (Note: IVIG is an immunomodulatory agent, not an immunosuppressant.)

Moreover, IVIG is generally safe during pregnancy, and it might be the better option for certain pregnant women with vasculitis. 

Lastly, IVIG may be used to counteract hypogammaglobulinemia caused by rituximab. 

Frequently Asked Questions

What to avoid if you have vasculitis?

Things to avoid if you have vasculitis:

• Processed foods
• Added sugars
• Alcohol
• Excessive salt
• Smoking 

What’s the life expectancy of people with vasculitis?

There’s no single answer. Moreover, we don’t have data on the link between specific types and lifespan. Some types are mild, while some are more severe. Types that affect vital organs like the brain, lungs, or kidneys can be life-threatening, particularly during the active disease state. Treatments that suppress the immune system may increase the risk of infections. With early diagnosis and modern treatments, many people with vasculitis live normal or near-normal lifespans.  

Can you recover from nerve damage caused by vasculitis?

Your chances of nerve recovery are higher if the treatment is started early. Sadly, in most cases, only partial recovery is possible. 

Should you follow a specific diet for vasculitis?

It’s hard to pinpoint and recommend a particular diet for a patient without knowing their unique individual situations. In general, it’s best to follow the American Heart Association’s dietary guidelines, which include:

• Plenty of whole grains, fruits, and vegetables
• Limited intake of added sugars, alcohol, and ultra-processed foods.