Types of Vasculitis Explained

Vasculitis is an autoimmune disease that leads to inflammation of the blood vessels. Vasculitis is a rare disorder in which inflammation causes the lining of the arteries to narrow. This decreases blood flow and can lead to organ dysfunction or damage.

Symptoms of vasculitis are characteristic of the types of blood vessels involved and can be mild such as causing a rash, joint pain and swelling, or severe leading to decreased organ function. Vasculitis is classified depending on the size of the blood vessels affected and may involve large, medium, or small arteries.

The following are the most common forms of vasculitis that rheumatologists treat:

Types of Vasculitis in the Large Vessels: Aorta and the Branches of the Aorta

Large arteries in the body transport blood from the heart to major organs and tissues. The largest artery in the body is called the aorta and the immediate branches from the aorta supply blood flow to the brain, heart, limbs, and abdominal organs. Rheumatologists treat Giant Cell Arteritis and Takayasu Arteritis.

Giant Cell Arteritis

The American College of Rheumatology defines giant cell arteritis (GCA) as vasculitis affecting the arteries in the head and scalp. These arteries are responsible for the blood supply to the brain, eyes, and face. Also known as “temporal arteritis”, this type of vasculitis is most common in individuals 50 years of age and older, and is the most common type of vasculitis in adults.

Common symptoms of giant cell arteritis include:

  • New headache or change in characteristic of existing headaches
  • Tenderness of the scalp or area over the temples (often tender to the touch)
  • Pain in the jaw or tongue with chewing
  • Abrupt onset of vision changes or vision loss, usually one eye at a time
  • Unexplained fever or weight loss
  • Asymmetric blood pressures

Giant cell arteritis causes several major arteries in the brain, face, or neck to narrow due to inflammation. This can lead to increased risk of stroke and vision loss.

Other symptoms of polymyalgia rheumatica (PMR) can also be present with GCA and are characterized by neck pain and bilateral shoulder and hip pain that typically comes on suddenly. PMR is not specifically vasculitis but is strongly associated with giant cell arteritis. Approximately 15% of individuals with PMR also have symptoms of GCA.

The most common treatment for giant cell arteritis (and polymyalgia rheumatica) is steroids, often on oral or IV form. Prednisone typically leads to rapid improvement in symptoms. Prednisone cannot reverse damage caused by stroke or reverse vision loss, but it does treat the inflammation within the blood vessels and decreases the risk of further damage.

Other treatments for GCA are tocilizumab (Actemra) and methotrexate; sarilumab (Kevzara) is approved for the treatment of polymyalgia rheumatica.

Takayasu Arteritis

Takayasu arteritis is the most rare form of vasculitis. Takayasu arteritis is caused by inflammation in the aorta and its immediate branches. This type of vasculitis is also called “pulseless disease” because some patients experience a weak or undetectable pulse in their arms or legs.

Takayasu arteritis can be difficult to diagnose because it is very rare and often doesn’t have many noticeable symptoms. Some Takayasu arteritis symptoms may include:

  • Extreme fatigue in the limbs with movement, especially with abrupt changes in position
  • Rapid weight loss
  • Unexplained fever
  • Extreme muscle pain or weakness
  • Numbness or tingling in a limb with activity that can be asymmetric (different on each side of the body)

Takayasu arteritis is usually diagnosed in younger individuals, often under 40. This type of vasculitis is often diagnosed between the ages of 20-40 and is most common in females of Asian descent. Treatment for Takayasu may include a combination of corticosteroids, immunosuppressants, and biologic medications.

Types of Vasculitis in the Medium Vessels

Medium vessels are the arteries that branch off the aorta (arterioles). There are 2 types of vasculitis associated with medium vessels.

Kawasaki Disease

Kawasaki disease is a type of vasculitis that causes inflammation in coronary arteries, or blood flow directly to the heart. This form of vasculitis is most common in children, particularly children under the age of 5.

Some of the common symptoms of Kawasaki disease include:

  • Swollen lymph nodes (mainly in the neck)
  • Fever
  • Rash and/or peeling skin
  • Swelling in the hands and feet
  • Red eyes, tongue, lips, mouth, or throat
  • Gastrointestinal distress

It is not known what causes Kawasaki disease, but it’s believed that the vasculitis may be triggered by bacterial infections or viruses affecting children. There may also be a genetic predisposition that triggers the disease.

Treatments for Kawasaki disease include IVIG infusions (intravenous gamma globulin). This treatment is often successful when given in the first several days of the onset of illness. NSAIDs (mainly Aspirin) are used to control fevers and reduce pain and swelling. Corticosteroids may also be used as a treatment for Kawasaki disease.

Polyarteritis Nodosa

Polyarteritis nodosa is a type of medium vessel vasculitis that can affect the entire body, but often presents with symptoms involving the digestive tract, joints, and nervous system. It is more common in men, but women can also develop this type of vasculitis.

Common symptoms of polyarteritis nodosa include:

  • Nerve symptoms, including tingling, numbness, and pain
  • Rashes
  • Muscle aches
  • Joint pain
  • GI distress or issues with digestion
  • Fever
  • High blood pressure
  • Loss of appetite and weight loss

Like other types of vasculitis, the cause of polyarteritis nodosa is not well understood. Polyarteritis nodosa is an autoimmune disorder, so it’s possible that it can be triggered by certain infections or environmental exposures. There is a connection between some individuals with polyarteritis nodosa and Hepatitis B, but the link is unclear.

Treatments for this disorder include steroids (like prednisone), and other immunosuppressants including cyclophosphamide.

Types of Vasculitis in the Small Vessels

Small-vessel vasculitis affects the smallest blood vessels (capillaries). These small vessels branch off the medium vessels and directly supply the organs, particularly the skin, kidneys, and lungs.

These types of vasculitis can be identified with an antibody or blood test called ANCA (antineutrophilic cytoplasmic antibody) which can be either positive or negative in individuals with these forms of vasculitis.

ANCA vasculitis includes several types of vasculitis:

  • Eosinophilic granulomatosis with polyangiitis (EPGA) or Churg-Strauss syndrome
  • Granulomatosis with polyangiitis (GPA) or Wegener’s Granulomatosis
  • Microscopic polyangiitis (MPA)
  • Renal-limited vasculitis or pauci-immune vasculitis

ANCA-associated vasculitis is the most prevalent type of small-vessel vasculitis. ANCAs are a type of antibody in the immune system that activate critical cells within the lining of the blood vessels. These types of vasculitis may cause issues with organ function because the network of these blood vessels is vast.

Symptoms of ANCA vasculitis vary depending on the specific type, but most often include:

  • Weight loss
  • Fever
  • Kidney and lung damage, sometimes coughing up blood
  • Nerve pain
  • Joint pain
  • Extreme fatigue
  • GI distress

The treatment for ANCA vasculitis includes a combination of corticosteroids (to reduce inflammation), and other immunosuppressants. There is no cure for ANCA vasculitis, but symptom management can help minimize chronic pain and organ damage.

Non-ANCA Vasculitis

Unlike ANCA vasculitis, non-ANCA vasculitis is not caused by an overproduction of ANCA antibodies. These syndromes are caused by other proteins in the immune system that destroy the membranes of blood vessels.

The primary type of non-ANCA vasculitis is Henoch-Schonlein purpura or IgA-mediated vasculitis. This type of vasculitis is primarily diagnosed in children under the age of 10, and can also be seen in adults. It rarely affects teens.

The following symptoms are often seen in IgA vasculitis:

  • Red/purple spotted rash or purpura
  • GI symptoms (diarrhea, vomiting, abdominal pain)
  • Swollen or painful joints
  • Kidney dysfunction

Like other types of vasculitis, the cause of Henoch-Schonlein purpura is not fully understood. It is believed that IgA vasculitis is triggered by certain environmental exposures, medications, viruses, or even exposure to cold temperatures.

IgA vasculitis is treated with corticosteroids and other immunosuppressants and the symptoms most often resolve quickly.

There are many different types of vasculitis, including types that are not mentioned above. The Vasculitis Foundation offers in-depth information on more rare forms, including Bechet’s Disease, cryoglobulinemia, and rheumatoid vasculitis.

Our team at Summit Rheumatology offers treatment for many types of vasculitis diagnosed in adults. Give us a call at (480) 494-2770 or send us a message to learn more and to schedule an appointment with one of our rheumatology specialists.

 

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