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, veins, capillaries, and blood vessels to narrow, which then decreases blood flow. Symptoms of vasculitis are characteristic of the types of blood vessels involved and can be mild such as causing a rash, or severe leading to organ dysfunction. Vasculitis is classified depending on the size of the blood vessels affected and involve large, medium, or small blood vessels.

Depending on the extent of inflammation, and the area that the inflammation occurs, the severity of symptoms may vary. There are more than 20 types of vasculitis and many of these are diagnosed and treated by rheumatologists. The following are the most common forms of the disease:

Types of Vasculitis in the Large Vessels (Aorta and Arteries that branch from 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. There are 3 types of vasculitis that are commonly associated with the large arteries.

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 supplying the brain, eyes, and face with blood. 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
  • Jaw or tongue pain 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 bilateral shoulder pain, neck pain, 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 and 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 rarest of the types of large vessel vasculitis. It causes 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
  • Rapid weight loss
  • Unexplained fever
  • Muscle pain or weakness
  • Numbness or tingling in a limb with activity

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

Types of Vasculitis in Medium Vessels

Medium vessels include the arteries that branch off the aorta. These include arterioles and veins. There are 2 types of vasculitis associated with medium vessels.

Kawasaki Disease

Kawasaki disease is a type of vasculitis that causes inflammation in the medium vessels throughout the body, but primarily the coronary vessels. The disease is also called Kawasaki syndrome or mucocutaneous lymph node syndrome. This form of vasculitis is primarily diagnosed in children under 5, however it has been diagnosed in older children.

Some of the common Kawasaki disease symptoms 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

Doctors don’t know for certain what causes Kawasaki disease, but it’s believed that it may be triggered by bacterial infections or viruses. There may also be a genetic component of 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 displays symptoms involving the digestive tract, joints, organs, heart, and nervous system. It is often diagnosed 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
  • Fever
  • High blood pressure
  • Loss of appetite and weight loss

Like other types of vasculitis, doctors don’t fully understand the cause of polyarteritis nodosa. 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 connection isn’t fully understood.

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

Types of Vasculitis in Small Vessels

Small-vessel vasculitis affects the veins and capillaries. These small vessels branch off of larger arteries and arterioles and are responsible for carrying blood back to the heart. Types of small-vessel vasculitis include ANCA and non-ANCA vasculitis.

ANCA 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

ANCA (antineutrophil cytoplasmic antibodies) vasculitis is the most prevalent type of small-vessel vasculitis. ANCAs are a type of antibody in the immune system that activate critical cells. People with ANCA vasculitis, ANCAs are overproduced and consistently activate the immune system, which damages tissues and causes swelling.

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

  • Weight loss
  • Feber
  • Kidney and lung damage
  • Nerve pain
  • Joint pain
  • Fatigue
  • GI distress

Treatments for ANCA may include a combination of corticosteroids (to reduce inflammation), and other immunosuppressants. There isn’t a cure for ANCA vasculitis, but symptom management can help minimize chronic pain or organ damage.

Non-ANCA Vasculitis

Unlike ANCA vasculitis, non-ANCA vasculitis is not caused by an overproduction of ANCA antibodies. The primary type of non-ANCA vasculitis is Henoch-Schonlein purpura. This type of vasculitis is primarily diagnosed in children under the age of 10 and causes the following symptoms:

  • Red/purple spotty rash
  • GI symptoms (diarrhea, vomiting, abdominal pain)
  • Swollen or painful joints
  • Kidney distress

Like other types of vasculitis, the cause of Henoch-Schonlein purpura isn’t fully understood. Researchers believe it may be triggered by certain environmental exposures, medications, viruses, or even exposure to cold temperatures.

Henoch-Schonlein doesn’t always require treatment, as patients often recover on their own. Sometimes, corticosteroids are prescribed to manage inflammation, and pain relievers or anti-inflammatories can help manage pain or joint inflammation.

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

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