Vasculitis
Synonym(s): Temporal Arteritis, Cranial Arteritis, Giant Cell Arteritis
Table of Contents
What is Vasculitis including Temporal Arteritis?
Is there any treatment?
What is the prognosis?
What research is being done?
What is Vasculitis including Temporal Arteritis?
Vasculitis is an inflammation of the blood vessel system, which includes the veins, arteries, and capillaries. Vasculitis may affect blood vessels of any type, size, or location, and therefore can cause dysfunction in any organ system, including the central and peripheral nervous systems. The symptoms of Vasculitis depend on which blood vessels are involved and what organs in the body are affected. The disorder may occur alone or with other disorders such as Temporal Arteritis. Temporal Arteritis (also called cranial or Giant Cell Arteritis) is an inflammation of the temporal artery (which runs over the temple, beside the eye). Symptoms of this disorder may include stiffness, muscle pain, fever, severe headaches, pain when chewing, and tenderness in the temple area. Other symptoms may include anemia, Fatigue, Weight Loss, shaking, vision loss, and sweats.
Is there any treatment?
Treatment for Vasculitis depends on the severity of the disorder and the individual's general health. Treatment may include cortisone or cytotoxic drugs. Other treatments may include plasmapheresis (the removal and reinfusion of blood plasma), intravenous gammaglobulin, and cyclosporin. Some cases of Vasculitis may not require treatment. Treatment for Temporal Arteritis and its associated symptoms generally includes corticosteroid therapy. Early detection of Temporal Arteritis and immediate treatment are essential to prevent vision loss.
What is the prognosis?
The prognosis for individuals with Vasculitis varies depending on the severity of the disorder. Mild cases of Vasculitis are generally not life-threatening, while severe cases (involving major organ systems) may be permanently disabling or fatal. The prognosis for individuals with Temporal Arteritis is generally good. With treatment, most individuals achieve complete remission, however vision loss may be irreversible.
What research is being done?
Several components of the NIH support research on Vasculitis and Temporal Arteritis. The ultimate goals of this research are to increase scientific understanding of these disorders and to find ways to prevent, treat, and cure them.
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Detailed Information
What Is Vasculitis?
Types of Vasculitis?
What Causes Vasculitis?
Who Is At Risk for Vasculitis?
What Are the Signs and Symptoms of Vasculitis?
How Is Vasculitis Treated?
What Is Vasculitis?
Vasculitis is an inflammation of the blood vessels in the body. In Vasculitis, the body’s immune system mistakenly attacks the body’s own blood vessels, causing them to become inflamed. Inflammation can damage the blood vessels and lead to a number of serious complications.Vasculitis can affect any of the body’s blood vessels. These include arteries, veins, and capillaries. Arteries are vessels that carry blood from the heart to the body’s organs, veins are the vessels that carry blood back to the heart, and capillaries are the tiny blood vessels that connect the small arteries and veins.
When a blood vessel becomes inflamed, it can:
- Narrow, making it more difficult for blood to get through
- Close off completely so that blood can’t get through at all (occlusion)
- In rare cases, stretch and weaken so much that it bulges (aneurysm) and may possibly burst (aneurysm rupture)
Figure A shows a normal artery with normal blood flow (the inset image shows a cross-section of the normal artery). Figure B shows an artery narrowed due to inflammation in the arterial wall, causing decreased blood flow (the inset image shows a cross-section of the inflamed artery). Figure C shows a totally occluded (blocked) artery due to inflammation and scarring in the arterial wall (the inset image shows a cross-section of the block artery). Figure D shows an artery containing an aneurysm.
The disruption in blood flow from inflammation can damage the body’s organs. Specific signs and symptoms depend on which organ has been damaged and the extent of the damage. Typical signs and symptoms of inflammation (fever, swelling, and a general sense of feeling ill) are common among people with Vasculitis.
Outlook
The outlook for people who have Vasculitis varies depending on both the type and severity of the Vasculitis. The course of a case of Vasculitis can be hard to predict. Treatment is often very effective if Vasculitis is diagnosed and treated early. Vasculitis may disappear or go into a period of remission. In some cases, it may be a chronic problem, requiring ongoing treatment, or it may come back (“flare”) after a period of remission. In more severe cases, treatment may not help, and Vasculitis can lead to disability or even death.
Much is still unknown about Vasculitis. There are many different types of Vasculitis, but overall it is a fairly rare condition in the general population. Additional research is needed to learn more about the various types of Vasculitis and their causes, treatments, and remission patterns.
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Types of Vasculitis
There are many different disorders that are classified as types of Vasculitis. Although all of these disorders include inflammation of the blood vessels, most differ from each other in terms of what populations of people and which organs they affect.Behcet’s Disease
Behcet’s disease can affect any part of the body because it can occur in vessels of any size or type. Commonly affected organs include the eyes, the mouth, and the genitals. Patients with this type of Vasculitis are often younger than those with other types, many times in their twenties and thirties. The HLA-B51 gene allele is often associated with Behcet’s disease, but not everyone with the gene will develop the disease. Behcet’s is more common in individuals of Mediterranean, Middle Eastern, or Far Eastern descent, but is usually not found in Blacks.
Buerger’s Disease
Buerger’s disease typically affects blood flow to the hands and feet. Buerger’s disease is also known as thromboangiitis obliterans and is more likely to occur in smokers than nonsmokers.
Central Nervous System Vasculitis
Central nervous system Vasculitis affects the brain and sometimes the spinal cord.
Churg-Strauss Syndrome
Churg-Strauss syndrome can affect many different organs, but it most commonly affects the lungs, skin, kidneys, and heart. Many people who are diagnosed with Churg-Strauss syndrome also have asthma. They may have had preexisting, newly diagnosed, or recently worsened asthma when diagnosed with Churg-Strauss syndrome.
Cryoglobulinemia
Common signs and symptoms of cryoglobulinemia (KRI-o-GLOB-u-luh-NE-me-uh) are a rash on the lower limbs, arthritis, and nerve damage. Type 1 cryoglobulinemia is often associated with lymphoma, while Type 2 cryoglobulinemia is often associated with hepatitis C infection.
Giant Cell Arteritis
Common symptoms of Giant Cell Arteritis include headache, fever, blurred vision, and pain in the jaw, shoulders, or hips. It is the most common form of Vasculitis that occurs in adults older than 50 years. It is more likely to occur in people of Scandinavian origin, but can occur in people of any race.
Henoch-Schonlein Purpura
Henoch-Schonlein purpura most commonly affects the skin, kidneys, joints, and stomach. It is the most common form of Vasculitis found in children, but can occur at any age. When seen in children, it often follows an upper respiratory infection. It is not usually a chronic disease, and full recovery is common.
Microscopic Polyangiitis
Microscopic polyangiitis (POL-e-an-je-i-tis) most commonly affects the kidneys, skin, and nerves, but it can affect any organ in the body.
Polyarteritis Nodosa
Polyarteritis nodosa (POL-e-ar-ter-i-tis no-DO-suh) most commonly affects the kidneys, skin, and nerves, but it can affect any organ in the body. It is most common in people in their thirties and forties. Men are twice as likely as women to get polyarteritis nodosa. In some cases, it is associated with chronic hepatitis B infection or a very specific type of leukemia known as hairy cell leukemia.
Polymyalgia Rheumatica
Polymyalgia rheumatica (POL-e-mi-AL-je-a ROO-ma-ti-kuh) most commonly affects the large joints in the body, such as the shoulders and hips. It often occurs along with Giant Cell Arteritis.
Rheumatoid Vasculitis
Rheumatoid Vasculitis can affect many different organs, including the eyes, skin, hands, and feet. It commonly occurs in patients who have severe rheumatoid arthritis.
Takayasu’s Arteritis
Takayasu (Tock-e-AH-sue) arteritis (ar-ter-I-tis) affects the aorta (the largest blood vessel in the body) and its branches, which carry blood away from the heart. It typically occurs in Asian women younger than 40.
Wegener’s Granulomatosis
Wegener’s granulomatosis (GRAN-u-lo-ma-TO-sis) can occur anywhere in the body. It most commonly affects the upper respiratory tract (nose, sinuses, and throat), lungs, and kidneys. It is most common in people who are middle-aged and older.
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What Causes Vasculitis?
Vasculitis is an inflammation of the blood vessels, but what causes the inflammation is often unknown. It is sometimes a side effect of the body’s immune system response to a recent or chronic infection. It also can be a side effect of the body’s response to a medicine. The body sometimes recognizes a medicine as a foreign substance, and may develop an “allergic reaction” to try to get rid of it.Vasculitis is sometimes linked to other diseases, such as:
- Autoimmune system diseases. These are diseases that the Vasculitis patient may have had for years, in which the body’s immune system mistakenly attacks the body itself. Examples include systemic lupus erythematosus (lupus), rheumatoid arthritis, and scleroderma.
- Blood cancers. These include leukemia and lymphoma.
Who Is At Risk for Vasculitis?
Vasculitis can affect anyone, including men, women, and people of all ages. Some types of Vasculitis are more likely to affect certain populations than others. Examples of populations that might be more affected by certain types of Vasculitis include smokers, children, young women, middle-aged adults, and people with chronic hepatitis B and/or C infections.Go to Detailed Info Question list
What Are the Signs and Symptoms of Vasculitis?
Vasculitis can have many different signs and symptoms depending on the type of Vasculitis and which organs are involved as well as how severely they are affected. With Vasculitis, any number of organs may be involved, so a patient can experience very few signs and symptoms or may be very sick.There are two types of signs and symptoms that are common among people with Vasculitis: those that affect the body in a general way (systemic) and those that affect specific organs or body systems.
Systemic
Systemic signs and symptoms are not specific to a particular part of the body, but affect a person overall, causing general aches, pains, and sickness. Common systemic symptoms include:
- Fever
- Loss of appetite
- Weight Loss
- Fatigue (feeling tired) and weakness
- General aches and pains
These signs and symptoms are specific to a particular organ or body system. The organs and body systems that can be affected include:
- Skin. People may experience a variety of skin changes, including purple or red spots. The changes may look like clusters of small dots, splotches, bruises, or hives. They may be itchy or painless.
- Joints. People can experience aches and arthritis if the joints are affected.
- Lungs. People may experience shortness of breath or even cough up blood. On a chest x ray, lung symptoms may look like pneumonia, even though they are not.
- Gastrointestinal tract. Ulcers in the mouth may be present in some types of Vasculitis. Also, abdominal pain or bloody diarrhea can occur in people with Vasculitis. In some severe cases, the intestines can rupture.
- Sinuses, nose, and ears. People may experience sinus infections, chronic middle ear infections, ulcers in the nose, or in certain cases there may be hearing loss.
- Eyes. People whose eyes are affected by Vasculitis may experience a blurring or loss of vision.
- Brain. People may experience headaches, confusion, changes in behavior, or strokes.
- Nerves. People may experience numbness, tingling, and weakness in various parts of the body. They also may experience symptoms in their limbs, such as loss of feeling or strength in the hands and feet or shooting pains in the arms and legs.
How Is Vasculitis Treated?
Most cases of Vasculitis are treated with prescription medicines.Goals of Treatment
The main goal of treatment is to stop the inflammation in the affected blood vessels. Most treatments do this by stopping the immune or inflammatory response that caused the Vasculitis to occur.
Specific Types of Treatment
There are two types of prescription medicines that are typically used to treat Vasculitis: corticosteroid medicines and cytotoxic medicines.
Corticosteroid Medicines
Corticosteroid medicines are often called steroids, though these are not the same kind of steroids that athletes have been reported to use. These medicines are used to reduce the inflammation in the blood vessels. Examples of corticosteroids that the doctor might prescribe include prednisone, prednisolone, and methylprednisolone.
Cytotoxic Medicines
Cytotoxic medicines are typically used to treat cancer, but certain drugs also can be used to treat Vasculitis. They may be prescribed in severe cases or in cases in which the patient did not respond to corticosteroids. Sometimes, they are prescribed along with corticosteroids. Cytotoxic medicines work by killing the cells that have caused the inflammation. Examples of these medicines include azathioprine and cyclophosphamide. Doses used for Vasculitis are usually lower than those used to treat cancer.
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Credit: National Institute of Health