- Also Known As:
- Behçet Disease
- Giant Cell Arteritis (GCA)
- Polymyalgia Rheumatica
- Takayasu Arteritis
- Buerger Disease
- Central Nervous System Vasculitis
- Kawasaki Disease
- Polyarteritis Nodosa
- Eosinophilic Granulomatosis with Polyangiitis (EGPA)
- Churg-Strauss Syndrome
- IgA Vasculitis (Henoch-Schönlein Purpura)
- Essential Mixed Cryoglobulinemia
- Hypersensitivity Vasculitis (Allergic Vasculitis Cutaneous Vasculitis or Leukocytoclastic Vasculitis)
- Microscopic Polyangiitis
- Granulomatosis with polyangiitis (GPA Wegener Granulomatosis)
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What is vasculitis?
Vasculitis is a general term referring to a rare group of conditions marked by inflammation of blood vessels, causing them to be weakened, thickened, narrowed (stenosis), enlarged (aneurysm), blocked (occlusion), or scarred. There are many types of vasculitis based on the size of the vessel affected and may or may not be associated with another underlying problem.
When there is no identifiable cause, the condition is called primary vasculitis. When an underlying cause can be identified, it is called secondary vasculitis. Secondary vasculitis may be triggered:
- By an allergic reaction to a medication
- In response to an infection such as hepatitis C
- As a result of an autoimmune disease, such as rheumatoid arthritis
- In some cases, as a complication of blood cell cancers such as leukemia and lymphoma
Vasculitis can affect any type of blood vessel, including veins, arteries, and capillaries. It can limit the circulation of blood, leading to tissue or organ damage. In some cases, a bulge in a weakened vessel, called an aneurysm, can occur. An aneurysm can be a life-threatening complication because it can rupture unexpectedly, leading to an emergency situation involving heavy internal bleeding.
There are many types of vasculitis, which are often classified according to the size of the affected blood vessels. The table below summarizes some types of vasculitis and their specific symptoms.
|Name||Blood Vessels Affected||Description and Symptoms|
|Behçet Disease||Medium, small||Marked by painful mouth and genital sores and eye inflammation but can affect other organs and tissues as well, including the skin, lungs, and joints; tends to affect men more than women; occurs most often in people ages 20 to 40; may be related to the gene HLA-B51, but not everyone with the gene gets the disease; it is rare in the U.S., where between 3 and 5 of 100,000 people are affected, but it is common in areas like Turkey, affecting 1 in 250 people.|
|Giant Cell Arteritis (GCA)||Large||Also called temporal arteritis because it involves inflammation of the temporal arteries along the sides of the head near the temples; associated with headaches, scalp tenderness, jaw pain, and blurred vision; most common type of vasculitis in adults over age 50; half of those with GCA also develop polymyalgia rheumatica|
|Polymyalgia Rheumatica||Large||Marked by pain and stiffness in the shoulders and hips; found mostly in those over age 50; 10-20% of people also develop giant cell arteritis|
|Takayasu Arteritis||Large, medium||Affects the largest blood vessel in the body – the aorta – and its branches; most cases occur in young women, especially Asian women, and it can start in childhood; general symptoms of malaise, fatigue, fever, arm weakness, joint pain, headaches, and loss of appetite|
|Buerger Disease||Small, medium||Marked by reduced blood flow to the hands and feet, resulting in numbness and tingling, and can lead to gangrene (tissue death); associated with cigarette smoking; usually affects men ages 20 to 40|
|Central Nervous System Vasculitis||Medium||Very rare type of vasculitis that affects only the central nervous system (brain and spinal cord); symptoms include headache, changes in mental function, and muscle weakness or paralysis; often associated with lupus, dermatomyositis, and rheumatoid arthritis|
|Kawasaki Disease||Mostly medium, but can affect any blood vessel||Rare form of vasculitis that affects children, especially those under age 5; causes a fever that does not respond to medication, redness of the mucous membranes (eyes, mouth), and skin rash, affects blood vessels and the heart, and causes enlarged lymph nodes; it is the second-leading cause of acquired heart disease in children because it causes inflammation of blood vessels in the coronary arteries. Multisystem Inflammatory Syndrome in Children (MIS-C) has been associated with COVID-19 and presents with Kawasaki-like symptoms.|
|Polyarteritis Nodosa||Medium||Very rare; affects the skin, heart, kidneys, intestinal tract, joints, and central nervous system; associated with fever, weight loss, muscle and joint pain as well as anemia; some cases may be linked to hepatitis B or C infections; other complications include aneurysms in arteries to the kidneys, liver, or GI tract.|
|Eosinophilic Granulomatosis with Polyangiitis, EGPA (Churg-Strauss Syndrome)||Small||Marked by asthma and a very high number of eosinophils (type of white blood cell); can also affect the lungs, digestive tract, skin, the heart, and the nervous system; associated with granulomas (lumps due to tissue inflammation)|
|IgA Vasculitis (Henoch-Schönlein Purpura)||Small||Most common type of vasculitis in children; usually follows an upper respiratory tract, throat or gastrointestinal infection; marked by abdominal pain, blood in the urine, and inflammation of blood vessels in the skin, causing purplish spots or patches (purpura) on the arms and legs; usually resolves on its own within a few weeks|
|Essential Mixed Cryoglobulinemia||Small||Proteins called cryoglobulins will clump together with exposure to the cold, restricting blood flow, marked by red or purple skin rashes (purpura) usually on the lower extremities; can cause joint pain, weakness, limb tingling/numbness, and kidney inflammation; often associated with chronic hepatitis C infection and occurs as an immune response to that infection|
|Hypersensitivity Vasculitis (Allergic Vasculitis, Cutaneous Vasculitis, or Leukocytoclastic Vasculitis)||Small||Affects the skin, usually red spots on the lower legs or lower back; often caused by allergic reaction to a medicine or infection; usually resolves by stopping the medicine or treating the infection; however, up to 50% of cases have no known cause (idiopathic); may be acute and self-limited, recurrent, or chronic and sometimes may affect organs such as the joints, digestive tract, and kidneys; in rare cases, kidney failure may occur.|
|Microscopic Polyangiitis||Small, medium||Affects vessels in various organs, including the kidneys, lungs, nervous system, and skin; general symptoms include fever, muscle aches, and weight loss while organ-specific symptoms can include coughing up blood; sometimes can progress quickly and lead to kidney failure|
|Granulomatosis with polyangiitis, GPA (Wegener Granulomatosis)||Small||Rare form of vasculitis that can affect the upper respiratory tract, lungs, kidneys, and other organs; granulomas can form in the lungs and cause damage; can occur at all ages, especially 40-60 year olds; general symptoms include fever, muscle aches, loss of appetite, and weight loss, while more specific symptoms can include shortness of breath, wheezing, runny nose, facial pain, and chronic sinusitis and ear infections.|
For more information on each of these conditions, see the websites of the Vasculitis Foundation and the Johns Hopkins Vasculitis Center. For more information on symptoms by type of vasculitis, see the National Heart, Lung, and Blood Institute: Vasculitis.
General Signs and Symptoms
Vasculitis is an illness that can affect the whole body (systemic), so signs and symptoms may be non-specific and include those typical of inflammation and decreased blood flow.
General signs and symptoms may include:
- Loss of appetite
- Weight loss
- Body aches
- Night sweats
- Numbness or weakness (nerve issues)
These symptoms may appear rapidly or may develop gradually over the course of several months. They can vary considerably from person to person. Depending on the type, vasculitis can cause signs and symptoms that are limited to specific areas of the body and/or organs. The size and specific type of blood vessels that are affected often lead to a typical set of symptoms for each form of vasculitis. See the table in the previous section for more details on these symptoms.
A tissue biopsy is the gold standard test for diagnosis of vasculitis. It involves taking a tissue sample from a blood vessel or affected organ. A pathologist uses a microscope to examine the tissue sample for signs of inflammation or damage. (For more details, see the article on Anatomic Pathology: Histopathology.)
Before taking a biopsy, which is invasive, certain laboratory and non-laboratory tests can be performed to help determine which organs may be involved. Several of these tests are also used to monitor treatment.
Antineutrophil cytoplasmic antibodies (ANCA) tests—these may be used to help detect and diagnose certain forms of autoimmune vasculitis, including granulomatosis with polyangiitis (Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome). Sometimes these tests may also be used to monitor treatment and/or detect a relapse of these conditions.
For more detailed information, including a table that summarizes some results that may be seen, read the article on Antineutrophil Cytoplasmic Antibodies (ANCA, MPO, PR3).
General laboratory tests may be used to help diagnose vasculitis and to monitor the condition and/or its treatment. Examples include:
- Complete blood count (CBC) – this test is used to look for complications of vasculitis and its treatment. It evaluates a person’s red blood cells and hemoglobin for anemia and checks the white blood cell count, which can be increased in infection or reduced following some treatments. Increased numbers of white blood cells are seen with some types of vasculitis, such as eosinophilic granulomatosis with polyangiitis, EGPA (Churg-Strauss Syndrome).
- C-reactive protein (CRP) – this test detects inflammation in the body.
- Erythrocyte sedimentation rate (ESR) – this test also detects the presence of inflammation and can be increased with several types of vasculitis, such as microscopic polyangiitis and granulomatosis with polyangiitis (Wegener granulomatosis).
- Creatinine and blood urea nitrogen (BUN) – these tests assess kidney function, which may be affected by vasculitis and/or its treatment.
- Liver panel – this group of tests assess liver function to determine if the vasculitis is affecting the liver. It may also be used to monitor for side effects of treatment.
- Urinalysis – this test looks for the presence of protein and red blood cells in the urine, which can indicate inflammation within the kidney.
- Complement – part of the inflammatory response; often reduced with vasculitis
- Cryoglobulins – may be positive with vasculitis
- Lung function tests – to determine if airflow is restricted when vasculitis is affecting the lungs (for more information on these, visit the National Heart, Lung, and Blood Institute webpage on Pulmonary Function Tests)
- Cardiac tests – EKG, echocardiography
- Imaging studies – chest x-ray, CT scan, MRI, abdominal ultrasound, angiography (for more on imaging studies, see the website RadiologyInfo.org)
- Nerve conduction studies or nerve biopsy may be useful if there are symptoms of numbness or tingling.
The treatment of vasculitis depends primarily on whether it is primary or secondary, the organs affected, and its severity. If an underlying cause can be identified (secondary vasculitis), treatment should be directed at the cause. Examples include withdrawal of a triggering drug or treatment of an underlying infection.
Since vasculitis results from an overactive immune system, treatment often involves drugs that suppress the immune system. The most commonly used drugs are corticosteroids, such as prednisone. When used long term, corticosteroids are associated with side effects such as weight gain, reduced bone density, and increased risk of diabetes and high blood pressure. In order to reduce the corticosteroid requirements and still control the disease, other immunosuppressive drugs such as cyclophosphamide, methotrexate, and azathioprine may be used.
Your healthcare practitioner will discuss your treatment options with you. The choice of drug should be decided after considering the severity of the disease. All immunosuppressive drugs carry an increased risk of infection and potential for decreased blood cell counts. Frequent monitoring of the complete blood count (CBC), liver function tests, and kidney function tests (creatinine, BUN, urinalysis) can help detect such side effects early.
Some types of vasculitis may require surgery to remove abnormal blood vessel bulges (aneurysms).
Sources Used in Current Review
(December 10, 2018) Vasculitis and Thrombophlebitis. Medscape. Available online at https://emedicine.medscape.com/article/1008239-overview. Accessed May 2020.
Forms of Vasculitis. Vasculitis Foundation. Available online at https://www.vasculitisfoundation.org/education/forms/. Accessed May 2020.
Buerger Disease. Genetic and Rare Diseases Information Center, National Institutes of Health. Available online at https://rarediseases.info.nih.gov/diseases/5969/buerger-disease. Accessed May 2020.
Vasculitis. National Organization for Rare Disorders. Available online at https://rarediseases.org/rare-diseases/vasculitis/. Accessed May 2020.
Diagnosing Vasculitis. Johns Hopkins Vasculitis Center. Available online at https://www.hopkinsvasculitis.org/vasculitis/diagnosing-vasculitis/. Accessed May 2020.
Vasculitis Treatments. Johns Hopkins Vasculitis Center. Available online at https://www.hopkinsvasculitis.org/vasculitis-treatments/. Accessed May 2020.
Harrison’s Principles of Internal Medicine, 20th ed (2018): The Vasculitis Syndromes.
(July 16, 2019) Granulomatosis with Polyangiitis (GPA, formerly called Wegener’s), Cleveland Clinic. Available online at https://my.clevelandclinic.org/health/diseases/4757-granulomatosis-with-polyangiitis-gpa-formerly-called-wegeners. Accessed May 2020.
(March 7, 2016) Central Nervous System Vasculitis. Cleveland Clinic. Available online at https://my.clevelandclinic.org/health/diseases/13205-central-nervous-system-vasculitis. Accessed May 2020.
(July 17, 2019) Polyarteritis Nodosa. Cleveland Clinic. Available online at https://my.clevelandclinic.org/health/diseases/13284-polyarteritis-nodosa. Accessed May 2020.
(May 14, 2020) Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with Coronavirus Disease 2019 (COVID-19). CDC. Available online at https://emergency.cdc.gov/han/2020/han00432.asp. Accessed May 2020.
Sources Used in Previous Reviews
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The Johns Hopkins Vasculitis Center. Symptoms of Vasculitis. Available online at http://www.hopkinsvasculitis.org/vasculitis/symptoms-vasculitis/. Accessed February 2011.
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