What Is Vasculitis?

Vasculitis is an autoimmune disorder that causes inflammation of the blood vessels. It can affect arteries, veins, and capillaries. This condition causes blood vessel walls to swell, which restricts blood flow to vital organs. In some cases, vasculitis can cause serious complications.

There are many different types of vasculitis, which can be triggered by infections, autoimmune disorders, or reactions to certain medications.

Vasculitis can be classified into several types based on the size of the blood vessels affected and the underlying cause.

• ANCA-Associated Vasculitis This includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA). This type of vasculitis affects the small vessels, such as capillaries, venules, and arterioles in any organ in the body; it’s commonly found in the skin, kidneys and nerves.

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• Behcet’s Disease Behcet’s disease causes blood vessel inflammation throughout the body, causing such symptoms as mouth sores, skin lesions, eye inflammation, and genital sores.

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• Buerger’s Disease Also called thromboangiitis obliterans, Buerger’s disease leads to reduced blood flow in the arteries and veins in the arms and legs and potentially to blood clots in the blood vessels. People with this condition almost always smoke cigarettes.

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• Central Nervous System (CNS) Vasculitis This type of vasculitis involves inflammation of the blood vessel walls in the brain or spine, which make up the CNS. It often occurs alongside other autoimmune conditions, or with an infection or another type of vasculitis.

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• Churg-Strauss Syndrome Now known as eosinophilic granulomatosis with polyangiitis (EGPA), this type of vasculitis most commonly affects the lungs, skin, gastrointestinal tract, heart, and nervous system. Early symptoms may include asthma, allergic reactions, and sinusitis.

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• Cryoglobulinemia This type of vasculitis is characterized by the presence of cryoglobulins, atypical proteins in the blood that can clump together, block blood flow, and damage the skin, joints, nerves, and organs, especially the kidneys and liver. Cryoglobulinemia can be triggered by cold temperatures, sometimes leading to lack of blood flow to the fingers and toes, which are more subject to cold.

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• Giant Cell Arteritis (GCA) Formerly called temporal arteritis, GCA is the most common form of vasculitis. It primarily affects white women and older adults (over 50), causing inflammation in the large arteries of the head and neck, which can lead to complications including blindness. GCA sometimes occurs along with polymyalgia rheumatica.

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• Henoch-Schonlein Purpura Also known as IgA vasculitis, this condition affects small blood vessels and typically involves the skin, joints, intestines, and kidneys. It typically causes a purple rash on the lower legs and buttocks and can cause abdominal pain and aching joints. It’s most common in children under 10 years old.

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• Kawasaki Disease This rare condition is most common between the ages of 6 months and 5 years. It causes inflammation in all the arteries but is most concerning when it affects the coronary arteries, which supply blood to the heart.

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• Takayasu Arteritis This form of vasculitis affects large arteries like the aorta, which can lead to arm or chest pain, high blood pressure, and eventually heart failure or stroke. It usually affects girls and women younger than 40 years old.

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• Wegener’s Vasculitis Now known as granulomatosis with polyangiitis, it affects the small blood vessels, most commonly in the upper respiratory tract, lungs, and kidneys.

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The general symptoms of most types of vasculitis include:

• Fever
• Headache
• Fatigue
• Weight loss
• General aches and pain

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Symptoms may be mild or severe and may happen only once or recur regularly. Some people may not have symptoms until the disease is very advanced.

The symptoms of vasculitis vary depending on where in the body the blood vessel swelling and impeded blood flow is happening. Other symptoms may include:

• Heart Fatigue, dizziness, lightheadedness, impaired breathing, and chest pain
• Lungs Chest pain, impaired breathing, asthma, and coughing up blood
• Central Nervous System Headache, impaired vision, nerve damage, and even stroke
• Extremities Pain, stiffness, numbness, discoloration, and weakness in the arms, legs, hands, and feet
• Kidneys Elevated blood pressure, weight loss, nausea, blood in urine
• Digestive Tract Abdominal pain, blood in stools, and weight loss.
• Skin Sores or lesions, rash, and discoloration, including purpura — a condition that causes red or purple spots on the skin or in the mucous membranes
• Eyes Eye pain, blurred vision, vision loss, and red eyes
• Ears Hearing issues, hearing loss, and ear pressure
• Nose Sinus problems, congestion, and nosebleeds

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Some people get vasculitis without any known cause or trigger (idiopathic), while others may have a condition or external factor that triggers it. Triggers may include the following:

• Infections Certain infections, such as hepatitis B and hepatitis C can trigger vasculitis.
• Medications Some drugs, including hydralazine, allopurinol, minocycline and propylthiouracil may trigger vasculitis.

Although anyone can get vasculitis, there are some factors associated with an increased risk.

• Genetics In some cases, genetic predisposition may increase the risk. Behcet’s disease, granulomatosis with polyangiitis, and Kawasaki disease sometimes run in families.
• Blood Cancers Blood cancers such as leukemia and lymphoma can cause vasculitis.
• Immune System Diseases Autoimmune conditions such as rheumatoid arthritis, lupus, and scleroderma are associated with a higher risk of vasculitis.
• Age Some types, such as giant cell arteritis, are more common in adults over age 50. Kawasaki disease is most common in children younger than 5.
• Gender Certain types of vasculitis, like Takayasu arteritis and giant cell arteritis are more prevalent in women. Buerger’s disease is more common in men.
• Drug Use Using cocaine and smoking tobacco may increase the risk for some types of vasculitis.

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A vasculitis diagnosis involves a combination of your medical history, a physical examination, and diagnostic tests. You may be referred to a rheumatologist, a type of specialist who treats inflammatory diseases.

Diagnostic tests may include:

• Blood Tests Elevated levels of inflammatory markers, including C-reactive protein, may suggest vasculitis. The presence of certain antibodies, such as anti-neutrophil cytoplasmic antibody (ANCA), in the blood may also indicate vasculitis. In addition, a complete blood cell count is done to look for any abnormalities.
• Imaging CT scans, MRIs, and ultrasounds can help identify inflamed or damaged blood vessels to help determine which blood vessels and organs are affected.
• Biopsy In some cases, a small sample of the affected tissue such as the skin, kidney, temporal artery on the scalp, or lung is removed to look for signs of vasculitis.
• Angiography In this type of imaging, a thin catheter is used to inject dye into an artery or vein, then X-rays are done of the area of interest. The dye makes it possible for the outline of the blood vessels to show up on the X-rays, which can be useful in identifying signs of inflammation.

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Medications

Steroids and immunosuppressive drugs are the main drug options for treating vasculitis.

Immunosuppressive Drugs These drugs suppress the immune system to prevent it from attacking blood vessels. Depending on the type of vasculitis, medications used may include the following:

• tocilizumab (Actemra)
• methotrexate (Trexall)
• azathioprine (Imuran, Azasan)
• rituximab (Rituxan)
• mycophenolate (CellCept)
• cyclophosphamide (Cytoxan)

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Examples of NSAIDS include:

• Aspirin
• Ibuprofen (Advil, Motrin)
• Naproxen (Aleve, Naprosyn)

Surgery and Plasmapheresis

Certain lifestyle and wellness habits may reduce the risk of vasculitis flare-ups or exacerbation.

• Regular Checkups Stay on top of health screenings, especially if you have a history of autoimmune disorders.
• Vaccination Stay up-to-date on vaccinations to reduce the risk of infections that could trigger vasculitis.
• Avoid Smoking Smoking is a known risk factor for some types of vasculitis.
• Healthy Lifestyle Maintain a healthful diet and get regular exercise.

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Vasculitis complications depend on the type and severity of your condition and how you respond to treatment. Some of the complications may be related to side effects of the prescription medications used to treat vasculitis.

Complications may include:

• Organ damage: Some types of vasculitis can be severe, causing damage to major organs such as the heart, kidneys, or lungs.
• Blood clots and aneurysms: A blood clot may form in a blood vessel, obstructing blood flow, which could cause a stroke. In rare cases, vasculitis will cause a blood vessel to weaken and bulge, forming an aneurysm.
• Vision loss or blindness: This may happen in giant cell arteritis.
• Infections: Some of the medications used to treat vasculitis suppress the immune system, which put you at a higher risk for infection.

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People with vasculitis are at a higher risk for aneurysm, heart attack, and stroke. People experiencing any of the following symptoms should go to the emergency room or call 911 right away.

Symptoms of a dissection or rupture caused by an aneurysm may include:

• Light-headedness
• Paleness
• Rapid heart rate
• Sudden, strong pain in the stomach, chest, or back, which can travel upward or downward

Symptoms of a heart attack may include:

• Mild or strong chest pain or discomfort of the chest or upper stomach area
• Back, shoulder, or jaw pain
• Pain down the arm
• Shortness of breath, nausea, vomiting, unusual tiredness

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Symptoms of stroke may include:

• Sudden numbness or weakness in the face, arm or leg, especially on one side of the body
• Sudden confusion, trouble speaking or understanding speech
• Loss of vision in one or both eyes
• Sudden trouble walking and issues with balance and coordination

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Vasculitis can coexist with other autoimmune conditions, inflammatory diseases, or infections like hepatitis B or C.

The Takeaway

• Vasculitis is a serious but treatable condition that involves inflammation of the blood vessels and can affect multiple organs.
• Early diagnosis and treatment are critical in managing vasculitis and preventing long-term damage.
• Medications and lifestyle changes, such as quitting smoking and seeing your healthcare provider as instructed, can limit the damage caused by the condition.