Vasculitis

What is vasculitis?

Vasculitis is a term used to describe several conditions that cause inflammation of the blood vessels. The condition causes changes to blood vessel walls—either thickening, narrowing, scarring, or weakening the barriers. These changes can cause various complications, but blood flow restriction is the most common. In some cases, this can result in tissue and organ damage.

This is a rare condition, but there are many types of vasculitis. Some are short-term, or acute; others are long-lasting, or chronic. Vasculitis most often occurs when the body’s immune system mistakenly attacks the blood vessels. This can happen as a reaction to a specific medicine, infection, or an underlying disease. In many cases, the exact cause isn’t known.

Because of this, many think that vasculitis is an autoimmune disorder. However, the disease’s exact cause remains unknown, so only some forms of the condition are classified as autoimmune conditions. Vasculitis can also be one part of preexisting autoimmune and rheumatic diseases, like rheumatoid arthritis and scleroderma.

What are the symptoms of vasculitis?

Vasculitis describes a variety of conditions, which means symptoms may be difficult to classify. Some forms of the disease have localized symptoms, while others are more systemic. In many cases, the organ effected will be the best place to observe vasculitis symptoms. The condition can occur in one or more organs, so it is important to know which symptoms are characteristic of which body parts.

• Joints: Vasculitis of the joints can range from aches and pains, sometimes without obvious swelling, to full-blown arthritis. Symptoms can include joint pain and swelling and are often accompanied by a skin rash.
• Lungs: Vasculitis in the lungs is best identified through a persistent cough, which is often accompanied by blood, shortness of breath, and a pneumonia-like appearance on a patient chest X-ray.
• Skin: The easiest skin vasculitis symptom to observe is a condition called palpable purpura, which is a skin rash composed of purple spots that results from the internal rupture and bleeding of small blood vessels. A doctor can typically feel these spots with their fingertips, and they are most often found on the legs.
• Eyes: Vasculitis can cause vision problems and blindness by affecting ocular blood vessels.
• Brain: Central nervous system vasculitis can cause strokes, mental disability, headaches, and difficulty with coordination.

How do you get vasculitis?

The cause of vasculitis isn’t fully known. Many researchers think some forms of the condition are related to a person’s ancestry and genetic makeup. Other types of vasculitis are a result of an immune system reaction. If your vasculitis is related to the immune system, it may have been caused by any of the following:

• A reaction to a certain drug
• Certain infections, such as hepatitis B and hepatitis C
• Blood cancers

Other immune system diseases, like lupus, scleroderma, and rheumatoid arthritis, can also trigger vasculitis in various parts of the body.

What are the types of vasculitis?

There are several types of vasculitis, each with its own set of symptoms and risks. However, it is easier to think about the types of vasculitis as being either systemic or limited. Some forms of the condition are localized in a specific part of the body, while others extend to blood vessels throughout several organs. The type of vasculitis a person has will be determined by the underlying cause, the location of the affected blood vessels, and the type or size of the blood vessels affected.

There are around a dozen types of vasculitis:

• Behcet’s disease, which is characterized by mouth and genital ulcers, as well as eye inflammation.
• Buerger’s disease, also known as Thromboangiitis Obliterans, which typically affects smokers and can impact blood flow to the hands and feet.
• Eosinophilic granulomatosis with polyangiitis, which is associated with nasal polyps, sinusitis, asthma, and elevated eosinophil counts. It typically affects the peripheral nerves, skin, kidneys, heart, and lungs.
• Cryoglobulinemia, which may be associated with a hepatitis C virus infection or paraproteinemias. It is characterized by recurring red dots on the lower extremities.
• Giant cell arteritis, known previously as temporal arteritis, is the most common type of vasculitis in North America. Characterized by fever, jaw and scalp pain, and headache, it affects large blood vessels and most often affects people over 50 years old.
• Henoch-Schönlein purpura, which is often caused by an upper respiratory tract infection.
• Microscopic polyangiitis, which is a systemic vasculitis that affects small and medium-sized blood vessels.
• Polyarteritis nodosa, which involves several different organ systems but is often focused on medium-sized arteries.
• Polymyalgia rheumatica, which is a painful syndrome characterized by localized stiffness and discomfort in the shoulders and hips. It typically occurs alongside giant cell arteritis.
• Rheumatoid vasculitis, which is caused by rheumatoid arthritis.
• Takayasu’s arteritis, which is a large-vessel vasculitis that targets the aorta and its major branches.
• Granulomatosis with polyangiitis, which involves the lungs, upper respiratory tract, and kidneys.

How is vasculitis diagnosed?

A vasculitis diagnosis typically begins with a collection of your personal medical history and a physical exam. If a doctor suspects vasculitis development, you may receive a blood test, a urine test, various imaging tests, a biopsy, and/or an angiography.

During the physical exam, the doctor will likely discuss the Birmingham Vasculitis Activity Score, or BVAS. This tool is designed to assess the disease in patients who may have different forms of vasculitis. While only a doctor should administer these questions, patients can get an understanding of the process through online tools.

How is vasculitis treated?

There is no known cure for this condition, which means vasculitis treatment centers on mitigating symptoms and controlling inflammation. Before recommending a treatment, however, your doctor will perform various diagnostic tests to better understand the underlying cause of the condition. With a better knowledge of your vasculitis, a doctor can then more effectively prescribe treatment.

Most vasculitis treatments include medications, like corticosteroids, which can help to control inflammation. In some cases, a doctor may recommend surgery for bulges and growths caused by vasculitis. Additionally, blocked arteries will typically require surgical treatment.

What is it like living with vasculitis?

A person’s experience living with vasculitis will be extremely personal. Some forms of the condition disappear with time, while others remain for the rest of an individual’s life. In most cases, patients should understand that many vasculitis treatment medications can cause side effects, including weight gain, weight loss, a suppressed immune system, and severe fatigue. Developing a robust support network, keeping a healthy lifestyle, and understanding your condition can all help to improve quality of life after a vasculitis diagnosis.

However, it is important to follow your prescribed treatment plan. If you have trouble with any medications, talk to your doctor about follow-up care or changing strategies. If your body responds well to vasculitis treatment, the condition may go into remission.