Types of Vasculitis

There are many types of vasculitis. Each type involves inflamed blood vessels. However, most types differ in whom they affect and the organs that are involved.

The types of vasculitis often are grouped based on the size of the blood vessels they affect.

Mostly Large Vessel Vasculitis

These types of vasculitis usually, but not always, affect the body’s larger blood vessels.

Behçet's Disease

Behçet's (beh-CHET'S) disease can cause recurrent, painful ulcers (sores) in the mouth, ulcers on the genitals, acne-like skin lesions, and eye inflammation called uveitis (u-ve-I-tis).

The disease occurs most often in people aged 20 to 40. Men are more likely to get it, but it also can affect women. Behçet's disease is more common in people of Mediterranean, Middle Eastern, and Far Eastern descent, although it rarely affects Blacks.

Researchers believe that a gene called the HLA-B51 gene may play a role in Behçet's disease. However, not everyone who has the gene gets the disease.

Cogan's Syndrome

Cogan's syndrome can occur in people who have a systemic (sis-TEH-mik) vasculitis that affects the large blood vessels, especially the aorta and aortic valve. The aorta is the main artery that carries oxygen-rich blood from the heart to the body.

A systemic vasculitis is a type of vasculitis that affects you in a general or overall way.

Cogan's syndrome can lead to eye inflammation called interstitial keratitis (in-ter-STISH-al ker-ah-TI-tis). The syndrome also can cause hearing changes, including sudden deafness.

Giant Cell Arteritis

Giant cell arteritis (ar-ter-I-tis) usually affects the temporal artery, an artery on the side of your head. This condition also is called temporal arteritis. Symptoms of this condition can include headaches, scalp tenderness, jaw pain, blurred vision, double vision, and acute (sudden) vision loss.

Giant cell arteritis is the most common form of vasculitis in adults older than 50. It's more likely to occur in people of Scandinavian origin, but it can affect people of any race.

Polymyalgia Rheumatica

Polymyalgia rheumatica (POL-e-my-AL-jah RU-ma-ti-kuh), or PMR, commonly affects the large joints in the body, such as the shoulders and hips. PMR typically causes stiffness and pain in the muscles of the neck, shoulders, lower back, hips, and thighs.

PMR usually occurs by itself, but 10–20 percent of people who have PMR also develop giant cell arteritis. Also, about half of the people who have giant cell arteritis may develop PMR.

Takayasu's Arteritis

Takayasu's (tock-e-AH-sues) arteritis affects medium- and large-sized arteries, particularly the aorta and its branches. The condition sometimes is called aortic arch syndrome.

Though rare, Takayasu's arteritis mainly affects teenage girls and young women. The condition is most common in Asians, but it can affect people of all races.

Takayasu's arteritis is a systemic disease. A systemic disease is one that affects you in a general or overall way.

Symptoms of Takayasu's arteritis may include tiredness and a sense of feeling unwell, fever, night sweats, sore joints, loss of appetite, and weight loss. These symptoms usually occur before other signs develop that point to arteritis.

Mostly Medium Vessel Vasculitis

These types of vasculitis usually, but not always, affect the body’s medium-sized blood vessels.

Buerger's Disease

Buerger's disease, also known as thromboangiitis (THROM-bo-an-jee-I-tis) obliterans, typically affects blood flow to the hands and feet. In this disease, the blood vessels in the hands and feet tighten or become blocked. As a result, less blood flows to the affected tissues, which can lead to pain and tissue damage.

Rarely, Buerger's disease also can affect blood vessels in the brain, abdomen, and heart. The disease usually affects men aged 20 to 40 of Asian or Eastern European descent. The disease is strongly linked to cigarette smoking.

Symptoms of Buerger's disease include pain in the calves or feet when walking or pain in the forearms and hands with activity. Other symptoms include blood clots in the surface veins of the limbs and Raynaud's phenomenon.

In severe cases, ulcers may develop on the fingers and toes, leading to gangrene. The term "gangrene" refers to the death or decay of body tissues.

Surgical bypass of the blood vessels may help restore blood flow to some areas. Medicines generally don’t work well to treat Buerger’s disease. The best treatment is to stop using tobacco of any kind.

Central Nervous System Vasculitis

Central nervous system (CNS) vasculitis usually occurs as a result of a systemic vasculitis. A systemic vasculitis is one that affects you in a general or overall way.

Very rarely, vasculitis affects only the brain and/or spinal cord. When it does, the condition is called isolated vasculitis of the central nervous system or primary angiitis (an-jee-I-tis) of the central nervous system.

Symptoms of CNS vasculitis include headaches, problems thinking clearly, changes in mental function, or stroke-like symptoms, such as muscle weakness and paralysis (an inability to move).

Kawasaki Disease

Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease in which the walls of the blood vessels throughout the body become inflamed. The disease can affect any blood vessel in the body, including arteries, veins, and capillaries.

Kawasaki disease also is known as mucocutaneous (myu-ko-kyu-TA-ne-as) lymph node syndrome. This is because the disease is associated with redness of the mucous membranes in the eyes and mouth, redness of the skin, and enlarged lymph nodes. (Mucous membranes are tissues that line some organs and body cavities.)

Sometimes the disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, a small number of children who have Kawasaki disease may have serious heart problems.

Polyarteritis Nodosa

Polyarteritis nodosa (POL-e-ar-ter-I-tis no-DO-suh) can affect many parts of the body. This disorder often affects the kidneys, the digestive tract, the nerves, and the skin.

Symptoms often include fever, a general feeling of being unwell, weight loss, and muscle and joint aches, including pain in the calf muscles that develops over weeks or months.

Other signs and symptoms include anemia (a low red blood cell count), a lace- or web-like rash, bumps under the skin, and stomach pain after eating.

Researchers believe that this type of vasculitis is very rare, although the symptoms can be similar to those of other types of vasculitis. Some cases of polyarteritis nodosa seem to be linked to hepatitis B or C infections.

Mostly Small Vessel Vasculitis

These types of vasculitis usually, but not always, affect the body’s small blood vessels.

Churg-Strauss Syndrome

Churg-Strauss syndrome is a very rare disorder that causes blood vessel inflammation. The disorder also is known as allergic angiitis and granulomatosis (GRAN-u-lo-ma-TO-sis).

Churg-Strauss syndrome can affect many organs, including the lungs, skin, kidneys, nervous system, and heart. Symptoms can vary widely. They may include asthma, higher than normal levels of white blood cells in the blood and tissues, and abnormal lumps known as granulomas (gran-yu-LO-mas).

Essential Mixed Cryoglobulinemia

Essential mixed cryoglobulinemia (KRI-o-GLOB-u-luh-NE-me-uh) can occur alone or with a systemic vasculitis. A systemic vasculitis is one that affects the body in a general or overall way.

“Cryoglobulinemia” refers to abnormal proteins in the blood. These proteins form a gel when a blood sample is exposed to temperatures lower than normal body temperature.

Symptoms often include joint aches; weakness; nerve changes, such as numbness, tingling, and pain in the limbs; kidney inflammation; and a raised, bumpy, reddish-purple skin rash known as palpable purpura (PURR-purr-ah).

Although essential mixed cryoglobulinemia can occur with other conditions, most often it’s linked to chronic hepatitis C infection.

Henoch-Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a type of vasculitis that affects the smallest blood vessels in the skin, joints, intestines, and kidneys. These blood vessels are called capillaries.

Symptoms often include stomach pain, aching and swollen joints, and signs of kidney damage, such as blood in the urine. Another symptom is a bruise-like rash that mostly shows up as reddish-purple blotches on the lower legs and buttocks (although it can appear anywhere on the body).

HSP is most common in children, but it also can affect teens and adults. In children, about half of all cases follow a viral or bacterial upper respiratory infection. Most people get better in a few weeks and have no lasting problems.

Hypersensitivity Vasculitis

Hypersensitivity vasculitis affects the skin. This condition also is known as allergic vasculitis, cutaneous (ku-TA-ne-us) vasculitis, or leukocytoclastic (LU-ko-si-TOE-klas-tic) vasculitis.

A common symptom is red spots on the skin, usually on the lower legs. For people who are bedridden, the rash appears on the lower back.

An allergic reaction to a medicine or infection often causes this type of vasculitis. Stopping the medicine or treating the infection usually clears up the vasculitis. However, some people may need to take anti-inflammatory medicines, such as corticosteroids, for a short time. These medicines help reduce inflammation.

Microscopic Polyangiitis

Microscopic polyangiitis (POL-e-an-jee-I-tis) affects small blood vessels, particularly those in the kidneys and lungs. The disease mainly occurs in middle-aged people; it affects men slightly more often than women.

The symptoms often aren't specific, and they can begin gradually with fever, weight loss, and muscle aches. Sometimes the symptoms come on suddenly and progress quickly, leading to kidney failure.

If the lungs are affected, coughing up blood may be the first symptom. Sometimes microscopic polyangiitis occurs with a vasculitis that affects the intestinal tract, the skin, and the nervous system.

The signs and symptoms of microscopic polyangiitis are similar to those of Wegener's granulomatosis (another type of vasculitis). However, microscopic polyangiitis usually doesn't affect the nose and sinuses or cause abnormal tissue formations in the lungs and kidneys.

The results of certain blood tests can suggest inflammation. These results include a higher than normal erythrocyte sedimentation rate (ESR); lower than normal hemoglobin (HEE-muh-glow-bin) and hematocrit (hee-MAT-oh-crit) levels (which suggest anemia); and higher than normal white blood cell and platelet (PLATE-let) counts.

Also, more than half of the people who have microscopic polyangiitis have certain antibodies (proteins) in their blood. These antibodies are called antineutrophil cytoplasmic autoantibodies (ANCA). ANCA also occur in people who have Wegener's granulomatosis.

Testing for ANCA can't be used to diagnose either of these two types of vasculitis. However, testing can help evaluate people who have vasculitis-like symptoms.

Wegener's Granulomatosis

Wegener's granulomatosis is a rare type of vasculitis. It affects men and women equally, but it occurs more often in Whites than in African Americans. This type of vasculitis can occur at any age, but it’s more common in middle-aged people.

Wegener's granulomatosis typically affects the sinuses, nose, and throat; the lungs; and the kidneys. The condition also can affect other organs.

In addition to inflamed blood vessels, the affected tissues also develop abnormal lumps called granulomas. If granulomas develop in the lungs, they can destroy the lung tissue. The damage can be mistaken for pneumonia or even lung cancer.

Symptoms of Wegener's granulomatosis often aren’t specific. They can begin slowly with fever, weight loss, and muscle aches. Sometimes the symptoms come on suddenly and progress rapidly, leading to kidney failure. If the lungs are affected, coughing up blood may be the first symptom.

The results of certain blood tests can suggest inflammation. These results include a higher than normal ESR; lower than normal hemoglobin and hematocrit levels (which suggest anemia); and higher than normal white blood cell and platelet counts.

Another test looks for antiproteinase-3 (an antineutrophil cytoplasmic autoantibody (ANCA)) in the blood. Most people who have active Wegener's granulomatosis will have this antibody. A small portion may have another ANCA known as antimyeloperoxidase-specific ANCA.

Having either ANCA antibody isn't enough on its own to make a diagnosis of Wegener's granulomatosis. However, testing for the antibodies can help support the diagnosis in patients who have other signs and symptoms of the condition.

A biopsy of an affected organ is the best way for your doctor to make a firm diagnosis. A biopsy is a procedure in which your doctor takes a small sample of your body tissue to examine under a microscope.

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