Vasculitis
Definition :
Vasculitis is an inflammation of your blood vessels. Vasculitis causes changes in the walls of your blood vessels, including thickening, weakening, narrowing and scarring.
There are many types of vasculitis. Some forms last only a short time (acute) while others are long lasting (chronic). Vasculitis, which is also known as angiitis and arteritis, can be so severe that the tissues and organs supplied by the affected vessels don't get enough blood. This shortage of blood can result in organ and tissue damage, even death.
Vasculitis can affect anyone, though some types of vasculitis are more common among certain groups. Some forms of vasculitis improve on their own, but others require treatment — often including taking medications for an extended period of time.
Symptoms:
General signs and symptoms common to most vasculitis types
The signs and symptoms of vasculitis vary depending on which blood vessels and, as a result, which organ systems are affected. However, general signs and symptoms that many people with vasculitis experience include:
Make an appointment with your doctor if you have any signs or symptoms that worry you.
Causes:
Vasculitis occurs when your immune system mistakenly sees blood vessel cells as foreign. The immune system then attacks those cells as if they were an invader, such as a bacteria or virus. It's not always clear why this happens, but an infection, some cancers, certain immune system disorders or an allergic reaction may serve as the trigger.
Blood vessels affected by vasculitis become inflamed, which can cause the layers of the blood vessel wall to thicken. This narrows the blood vessels, reducing the amount of blood — and therefore oxygen and vital nutrients — that reaches your body's tissues. In some cases, a blood clot may form in an affected blood vessel, obstructing blood flow. Sometimes instead of becoming narrower, a blood vessel may weaken and form a bulge (aneurysm), a potentially life-threatening condition.
Vasculitis with no known cause (primary vasculitis)
For many of its forms, the cause of vasculitis is unknown. These forms of vasculitis are called primary vasculitis.
Vasculitis that occurs due to another disease (secondary vasculitis)
Forms of vasculitis for which an underlying disease is the cause are called secondary vasculitis. Examples of causes of secondary vasculitis include:
Complications of vasculitis depend on the type of vasculitis you have. In general, complications that can occur include:
Specific treatment for vasculitis depends on what kind of vasculitis you have, how serious your condition is and your general health. Though some types of vasculitis are self-limiting and improve on their own, such as Henoch-Schonlein purpura, others require medications.
Medications used to treat vasculitis include:
Definition :
Vasculitis is an inflammation of your blood vessels. Vasculitis causes changes in the walls of your blood vessels, including thickening, weakening, narrowing and scarring.
There are many types of vasculitis. Some forms last only a short time (acute) while others are long lasting (chronic). Vasculitis, which is also known as angiitis and arteritis, can be so severe that the tissues and organs supplied by the affected vessels don't get enough blood. This shortage of blood can result in organ and tissue damage, even death.
Vasculitis can affect anyone, though some types of vasculitis are more common among certain groups. Some forms of vasculitis improve on their own, but others require treatment — often including taking medications for an extended period of time.
Symptoms:
General signs and symptoms common to most vasculitis types
The signs and symptoms of vasculitis vary depending on which blood vessels and, as a result, which organ systems are affected. However, general signs and symptoms that many people with vasculitis experience include:
- Fever
- Fatigue
- Weight loss
- Muscle and joint pain
- Loss of appetite
- Nerve problems, such as numbness or weakness
- Behcet's syndrome. This condition causes
inflammation of your arteries and veins, and often appears in your 20s
and 30s. Signs and symptoms include mouth and genital ulcers, eye
inflammation, and acne-like lesions on your skin.
- Buerger's disease. Also called thromboangiitis
obliterans, this condition causes inflammation and clots in the blood
vessels in your extremities. Signs and symptoms can include pain in your
hands, arms, feet and legs, and ulcers on your fingers and toes. This
disorder is strongly associated with cigarette smoking.
- Churg-Strauss syndrome. This condition, also known
as allergic granulomatosis and allergic angiitis, most commonly affects
the blood vessels in your lungs. It's often associated with asthma.
- Cryoglobulinemia. This condition is often associated with hepatitis C infections. Signs and symptoms include a rash called purpura on your lower extremities, arthritis, weakness and nerve damage (neuropathy).
- Giant cell arteritis. This condition, which usually
occurs in people older than 50, is an inflammation of the arteries in
your head, especially your temples. Giant cell arteritis can cause
headaches, scalp tenderness, jaw pain while chewing, blurred or double
vision, and even blindness. Giant cell arteritis is often associated
with another type of inflammatory condition called polymyalgia
rheumatica (PMR). PMR causes pain in and inflammation of the large
joints, such as your shoulders and hips. Signs and symptoms include pain
and stiffness in the muscles of your hips, thighs, shoulders, upper
arms and neck.
- Henoch-Schonlein purpura. This condition is caused
by inflammation of the blood vessels of your skin, joints, bowel and
kidneys. Signs and symptoms can include abdominal pain, blood in the
urine, joint pain, and a rash called purpura on your buttocks, legs and
feet. Henoch-Schonlein usually affects children, but it can occur at any
age.
- Hypersensitivity vasculitis. The primary sign of
hypersensitivity vasculitis is red spots on your skin. It can be
triggered by an allergy, most often to a medication or an infection.
- Kawasaki disease. Also known as
mucocutaneous lymph node syndrome, this condition most often affects
children younger than 5 years of age. Signs and symptoms include fever,
skin rash and eye inflammation.
- Microscopic polyangiitis. This form of vasculitis
affects small-sized blood vessels in your kidneys, lungs and skin. Signs
and symptoms include skin lesions, fever, unintentional weight loss,
glomerulonephritis — inflammation of the small blood vessels in the
kidneys — and nerve damage.
- Polyarteritis nodosa. This form of vasculitis
affects medium-sized blood vessels in many different parts of the body,
including your skin, heart, kidneys, peripheral nerves, muscles and
intestines. Signs and symptoms include a rash called purpura, skin
ulcers, muscle and joint pain, abdominal pain, and kidney problems.
- Takayasu's arteritis. This form of vasculitis
includes the largest arteries in the body, including the aorta, and
typically occurs in young women. Signs and symptoms include a feeling of
numbness or cold in the extremities, decreased or absent pulses, high
blood pressure, headaches, and visual disturbances.
- Granulomatosis with polyangiitis (Wegener's). Formerly known as Wegener's granulomatosis, granulomatosis with polyangiitis causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Signs and symptoms can include nasal stuffiness, chronic sinus infections and nosebleeds. The kidneys are often affected, though most people won't have any noticeable symptoms until the damage is more advanced.
Make an appointment with your doctor if you have any signs or symptoms that worry you.
Causes:
Vasculitis occurs when your immune system mistakenly sees blood vessel cells as foreign. The immune system then attacks those cells as if they were an invader, such as a bacteria or virus. It's not always clear why this happens, but an infection, some cancers, certain immune system disorders or an allergic reaction may serve as the trigger.
Blood vessels affected by vasculitis become inflamed, which can cause the layers of the blood vessel wall to thicken. This narrows the blood vessels, reducing the amount of blood — and therefore oxygen and vital nutrients — that reaches your body's tissues. In some cases, a blood clot may form in an affected blood vessel, obstructing blood flow. Sometimes instead of becoming narrower, a blood vessel may weaken and form a bulge (aneurysm), a potentially life-threatening condition.
Vasculitis with no known cause (primary vasculitis)
For many of its forms, the cause of vasculitis is unknown. These forms of vasculitis are called primary vasculitis.
Vasculitis that occurs due to another disease (secondary vasculitis)
Forms of vasculitis for which an underlying disease is the cause are called secondary vasculitis. Examples of causes of secondary vasculitis include:
- Infections. Some vasculitis occurs in response to
an infection. For instance, most cases of cryoglobulinemia are the
result of the hepatitis C virus infection, and the hepatitis B virus
infection causes some cases of polyarteritis nodosa.
- Immune system diseases. Vasculitis can also occur
as the result of some diseases of the immune system, such as rheumatoid
arthritis, lupus and scleroderma.
- Allergic reactions. Sometimes an allergic reaction to a medication may cause vasculitis.
- Blood cell cancers. Cancer that affects the blood cells, including leukemia and lymphoma, can cause vasculitis.
Complications of vasculitis depend on the type of vasculitis you have. In general, complications that can occur include:
- Organ damage. Some types of vasculitis can be severe, causing damage to major organs.
- Recurring episodes of vasculitis. Even when treatment for vasculitis is initially successful, the condition may recur and require additional treatment. In other cases, vasculitis may never completely go away and requires ongoing treatment.
Specific treatment for vasculitis depends on what kind of vasculitis you have, how serious your condition is and your general health. Though some types of vasculitis are self-limiting and improve on their own, such as Henoch-Schonlein purpura, others require medications.
Medications used to treat vasculitis include:
- Steroids to control inflammation. Treatment for
many types of vasculitis consists of doses of a corticosteroid drug,
such as prednisone or methylprednisolone (Medrol), to control
inflammation. Side effects of steroids can be severe, especially when
taken over a long period of time. Side effects can include weight gain,
diabetes and bone thinning (osteoporosis). You're likely to receive the
lowest dose of steroids possible to control your disease.
- Medications to control the immune system. Severe cases of vasculitis or those that don't respond adequately to corticosteroids may need treatment with cytotoxic drugs that kill immune system cells responsible for causing inflammation. Cytotoxic drugs include azathioprine (Azasan, Imuran) and cyclophosphamide (Cytoxan). Another drug that helps dampen the immune system response is rituximab (Rituxan). This drug is approved for treating granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis along with corticosteroids. Researchers have also had some success using drugs that alter your body's immune response to treat certain types of vasculitis. Drugs that have been used, but are still being tested include mycophenolate (Cellcept), infliximab (Remicade), adalimumab (Humira) and anakinra (Kineret).
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