Vasculitis

Vasculitis is a general term for a group of uncommon diseases that feature inflammation of the blood vessels.

The blood vessels in the body are part of the circulatory system and include arteries that carry blood from the heart to the organs of the body and veins that return blood to the heart.

Vasculitis describes disease that damages blood vessels due to inflammation of the blood vessel wall (vasc=vessel + itis=inflammation). White blood cells invade the walls of the blood vessel causing an inflammatory reaction that results in swelling and damage.

Vasculitis may be a primary disease that occurs by on its own or a secondary condition related to another underlying illness.

In severe cases, the blood vessel damage can cause bleeding at the site of inflammation, or even narrow the blood vessel and decrease blood flow to the organs that it supplies. Both situations may also occur together.

The cause of vasculitis is usually not known. It may occur on its own, or may be associated with other conditions or diseases.

Immune system abnormality and inflammation of blood vessels are common features. Each form of vasculitis has its own characteristic pattern of symptoms, much of which depends on what particular organs are affected.

Risk factors for developing vasculitis may include:

• Age: Some types of vasculitis occur more often in people at certain ages
• Genetics: Some types of vasculitis may run in families
• Ethnicity: Certain types of vasculitis are more prevalent in some populations
• Gender: Some kinds of vasculitis are more common in one gender
• Lifestyle habits
  • Smoking
  • Use of certain illegal drugs such as cocaine
• Use of some medications
  • Allopurinol
  • Hydralazine
  • Levamisole
  • Propylthiouracil
  • Tumor necrosis factor (TNF) inhibitors
• Other medical conditions
  • Autoimmune disorders
  • COVID-19
  • Multisystem inflammatory syndrome (MIS-C)
  • Hepatitis B or C
  • Lymphoma

The classification of vasculitis is based upon whether the disease is limited only to the blood vessels and the size of the blood vessels involved. There is some disagreement regarding the classification systems for vasculitis. The European Alliance of Associations for Rheumatology and the American College of Rheumatology published updated classification criteria in 2022.

• Primary non-infectious vasculitis
  • Large vessel
    • Takayasu arteritis affects the aorta
    • Giant cell arteritis usually involves the temporal artery of the scalp but can also involve the aorta and the carotid arteries
  • Medium vessel
    • Polyarteritis nodosa
    • Kawasaki disease is most often seen in children where the coronary arteries can be involved (arteries to the heart muscle)
  • Small vessel
    • Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis
    • Microscopic polyangiitis
    • Granulomatosis with polyangiitis
    • Eosinophilic granulomatosis with polyangiitis 
    • Immune complex small vessel vasculitis
    • Anti-glomerular basement membrane disease affects the kidney
    • Cryoglobulinemic vasculitis describes a vasculitis that is brought on by cold exposure
    • Immunoglobulin A vasculitis, also known as Henoch Schoenlein purpura
  • Variable vessel
    • Bechet syndrome
    • Cogan syndrome
• Single organ vasculitis
  • causes inflammation localized to a single organ, but may later spread to other body parts and may then be reclassified
• Vasculitis associated with systemic disease
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
• Vasculitis associated with a probable cause.
  • An example includes hepatitis B virus-associated polyarteritis nodosa

Symptoms of vasculitis depend upon the organs that are involved and the severity of the condition.

Vasculitis may cause symptoms involving the whole body like fatigue, malaise, weakness, and weight loss.

Specific organ complaints may include the following:

• Skin: Rashes, skin discoloration, and ulcers
• Muscles: Muscle pain and tenderness
• Lung: Shortness of breath and cough
• Heart: Congestive heart failure
• Brain: Headaches, confusion, seizures, stroke, paralysis, numbness, and lightheadedness
• Kidney: Kidney failure

The diagnosis of vasculitis may often be difficult. Because different organs can be involved, there is no one question to ask the patient or one finding on physical exam. The health care provider needs to take the time to consider the potential diagnosis of a vasculitis and then consider testing to prove that diagnosis.

Blood tests often look for signs of inflammation and include ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein). Other screening tests will include a CBC (complete blood count), kidney function, and liver function.

Additional tests may include a chest X-ray, electrocardiogram (EKG), and electromyography (EMG).

Screening tests for rheumatoid arthritis (rheumatoid factor) and systemic lupus erythematosus (complement) may be considered.

The diagnosis of vasculitis can be definitively established with a biopsy of tissue that confirms a pattern of blood vessel inflammation. Examples of tissues used for biopsy include skin, lungs, nerves, and kidneys. Some parts of the body cannot be biopsied, like the aorta.

Other ways of evaluating blood vessels without biopsy include ultrasound, and CT and MRI scans with angiography.

The treatment of vasculitis begins with decreasing the present inflammation. Organs that are secondarily involved may need to be supported while that initial treatment becomes effective.

The first phase of treatment usually involves corticosteroids to aggressively control the inflammation. Other medications may also be used depending upon the disease.

Once the disease flare is under control, maintenance medications may be started to prevent recurrence. Over time, those maintenance medications may be weaned to lower doses and the patient will need to be monitored to make certain that there is no recurrence. These medications can have significant side effects and their use should be monitored.

Usually, the treatment and monitoring of vasculitis is lifelong.

Corticosteroids, such as prednisone, are commonly used to initially treat inflammation.

Medications like cyclophosphamide, methotrexate, rituxumab, and others, may also be considered, depending upon the underlying diagnosis.

Vasculitis can affect almost any organ and the failure of that organ is a possibility. A few examples include the following:

• Heart, lung, kidney, and liver can be involved with their subsequent failure to function normally.
• Peripheral nerve involvement can cause numbness and pain in the hands and feet.
• There can be bleeding and ulceration of the skin.
• The stomach and intestine can be affected with ulcer formation and bleeding.

Vasculitis involves a wide spectrum of diseases and involves many organ systems. The prognosis for vasculitis as a whole is uncertain and depends upon the type of vasculitis present. Some diseases are aggressive while others can be controlled with medications.

It is important to remember that some of the medications used can cause significant side effects.

The cause of vasculitis is not yet known and may kinds of vasculitis cannot be prevented. However, it may be possible to reduce the risk symptoms of vasculitis and prevent flare-ups:

• Medications
  • Anticoagulants
  • Beta blockers
  • Statins
• Lifestyle changes
  • A heart-healthy lifestyle
  • Don’t smoke
  • Don’t use illegal drugs