Vasculitis (vas-kyu-LI-tis) is a condition that involves inflammation in the blood vessels. The condition occurs if your immune system attacks your blood vessels by mistake. This may happen as the result of an infection, a medicine, or another disease or condition.
The inflammation can lead to serious problems. Complications depend on which blood vessels, organs, or other body systems are affected.
Vasculitis can affect any of the body`s blood vessels. These include arteries, veins, and capillaries. Arteries carry blood from your heart to your body`s organs. Veins carry blood from your organs and limbs back to your heart. Capillaries connect the small arteries and veins.
When a blood vessel is inflamed, it can narrow or close off. This limits or prevents blood from getting through the vessel. Rarely, the blood vessel will stretch and weaken, causing it to bulge. This bulge is known as an aneurysm (AN-u-rism).
The disruption in blood flow from inflammation can damage the body`s organs. Signs and symptoms depend on which organs have been damaged and the extent of the damage.
Typical symptoms of inflammation, such as fever and general aches and pains, are common among people who have vasculitis.
There are many types of vasculitis, but overall the condition is rare. If you have vasculitis, the outlook depends on:
The type of vasculitis you have
Which organs are affected
How quickly the condition worsens
How severe the condition is
Treatment often works well if the condition is diagnosed and treated early. In some cases, vasculitis may go into remission. `Remission` means the condition isn`t active, but it can come back, or `flare,` at any time.
Some cases of vasculitis are chronic (ongoing) and never go into remission. Long-term treatment with medicines often can control the signs and symptoms of chronic vasculitis.
Rarely, vasculitis doesn`t respond well to treatment. This can lead to disability and even death.
Much is still unknown about vasculitis. However, researchers continue to learn more about the condition and its various types, causes, and treatments.
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 larger blood vessels.
Beh et`s Disease
Beh et`s (beh-CHET`S) disease can cause recurrent, painful ulcers 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. It`s 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 Beh et`s disease. However, not everyone who has the gene gets the disease.
Cogan`s syndrome can occur in people who have a systemic vasculitis that affects the large 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). It 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 headache, 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 (POL-e-my-AL-ja 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.
Most often, PMR 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 also can have PMR.
Takayasu`s (tock-e-AH-sues) arteritis affects medium- and large-sized arteries, particularly the aorta and its branches. The condition is sometimes called aortic arch syndrome.
Though rare, Takayasu`s arteritis occurs mostly in teenage girls and young women. The condition is more common in Asians, but it can affect people of all races and occur throughout the world.
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 medium-sized blood vessels.
Buerger`s disease, also known as thromboangiitis 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. This causes less blood to flow 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 aren`t effective treatments. 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 CNS or primary angiitis of the CNS.
Symptoms of CNS vasculitis are headache, problems thinking clearly or changes in mental function, or stroke-like symptoms, such as muscle weakness and paralysis (an inability to move).
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 lymph node syndrome.
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 develop serious heart problems.
Polyarteritis nodosa (POL-e-ar-ter-I-tis no-DO-suh) can affect many parts of the body. It 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 abdominal 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 small blood vessels.
Churg-Strauss syndrome is a very rare disorder that causes blood vessel inflammation. It`s also known as allergic angiitis and granulomatosis (GRAN-u-lo-ma-TO-sis).
This disorder 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 tissue formations known as granulomas.
Essential Mixed Cryoglobulinemia
Essential mixed cryoglobulinemia (KRI-o-GLOB-u-luh-NE-me-uh) can occur alone, or it may be linked to a systemic vasculitis. A systemic vasculitis is one that affects the body in a general or overall way.
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).
While essential mixed cryoglobulinemia can occur with other conditions, it most often is linked to chronic hepatitis C infection.
Henoch-Sch nlein Purpura
Henoch-Sch nlein purpura (HSP) is a type of vasculitis that affects the smallest blood vessels the capillaries in the skin, joints, intestines, and kidneys.
Symptoms often include abdominal pain, aching and swollen joints, and signs of kidney damage, such as blood in the urine. Another sympto...