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This article waslast modified on October 30, 2019.
What is vasculitis?

Vasculitis refers to a rare group of conditions characterized by inflammation of blood vessels. Most commonly, in the absence of an identifiable cause, it 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, or as a result of an autoimmune disease, such as rheumatoid arthritis. In some cases, secondary vasculitis is seen 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 result in narrowed or blocked vessels that 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, leading to heavy internal bleeding.

There are many types of vasculitis, which are often classified according to the size of the affected blood vessels. On the next page is a table containing examples of types of vasculitis.

Accordion Title
About Vasculitis
  • Types and Symptoms of Vasculitis

    Because vasculitis is a systemic illness, symptoms can be non-specific and include those typical of inflammation, such as:

    • Fatigue
    • Loss of appetite
    • Weight loss
    • Fever
    • Body aches

    These symptoms may appear rapidly or may develop gradually over the course of several months. The presentation of symptoms can vary considerably from person to person. In addition to these diffuse symptoms, someone can have organ-specific, localized symptoms associated with the particular type of vasculitis that person has. The size and specific blood vessels that are affected often lead to a characteristic set of symptoms for each type. The table below provides examples of some types of vasculitis and their specific symptoms.

    Name Blood Vessels Affected Description and Symptoms
    Behçet Disease Large, medium, and small Characterized 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
    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 Characterized 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 Medium Characterized by reduced blood flow to the hands and feet, resulting in numbness and tingling, and can lead to gangrene; associated with cigarette smoking; usually affects men ages 20 to 40
    Central Nervous System Vasculitis Medium Very rare type of vasculitis in which involvement is confined to the central nervous system (brain and spinal cord); symptoms include headache, changes in mental function, and muscle weakness or paralysis
    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), skin rash, and enlarged lymph nodes
    Polyarteritis Nodosa Medium Affects the skin, heart, kidneys, and central nervous system; associated with fever, weight loss, muscle and joint pain as well as anemia; very rare, though the symptoms can be similar to those of other types of vasculitis; some cases may be linked to hepatitis B or C infections
    Eosinophilic Granulomatosis with Polyangiitis, EGPA (Churg-Strauss Syndrome) Small Characterized by inflammation of the nose, asthma, and skin and lung involvement, but can also affect other organ systems; very high number of eosinophils (type of white blood cell) may be found in blood and tissues; 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; characterized 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 Often associated with chronic hepatitis C infection and occurs as an immune response to that infection; characterized by purpura usually on the lower extremities; can cause joint pain, weakness, limb tingling/numbness, and kidney inflammation
    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 are idiopathic (have no known cause). It may be acute and self-limited, recurrent, or chronic and sometimes may affect organs such as the joins, gastrointestinal tract, and kidneys
    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 involve the upper respiratory tract, lungs, kidneys, and other organs; granulomas can occur in the lungs and cause damage; general symptoms include fever, muscle aches, and weight loss while more specific symptoms can include shortness of breath and chronic sinusitis and ear infections

    For more information on each of these conditions, see the web sites 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: Types of Vasculitis

  • Tests

    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 and examining it 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 determine which organs are involved. Several of these tests are also used to monitor treatment.

    Laboratory tests

    • 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 in several types of vasculitis, such as microscopic polyangiitis and granulomatosis with polyangiitis (Wegener granulomatosis).
    • Creatinine – this test assesses kidney function, which may be affected by vasculitis.
    • Liver panel – these tests assess liver function to determine if the vasculitis is affecting the liver.
    • Urinalysis – this test looks for the presence of protein and red blood cells in the urine, which can indicate inflammation within the kidney.
    • Anti-neutrophil cytoplasmic antibody (ANCA) – this test is a useful marker for some systemic vasculitis conditions, such as granulomatosis with polyangiitis and microscopic polyangiitis.
    • Complement – part of the inflammatory response; often reduced with vasculitis

    Non-laboratory tests

    • Lung function tests – to determine if airflow is restricted when vasculitis is affecting the lungs
    • Cardiac tests – EKG, echocardiography
    • Imaging studies – chest x-ray, CT scan, MRI, abdominal ultrasound, angiography
    • Nerve conduction studies or nerve biopsy may be useful if there are symptoms of numbness or tingling.

    For more on imaging studies, see the web site

  • Treatment

    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 and methylprednisolone. 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.

    The choice of drug for each patient should be decided after considering the severity of the disease. All immunosuppressive drugs carry an increased risk of infection and potential for reduction in blood cell counts. Frequent monitoring of the complete blood count (CBC), liver function tests, and kidney function tests (creatinine, BUN, urinalysis) can detect such side effects early.

    Some types of vasculitis may require surgery to remove abnormal blood vessel bulges (aneurysms).

View Sources

Sources Used in Current Review

(Reviewed 2014 April 1). Vasculitis. MedlinePlus. Available online at Accessed on 6/07/15.

(2014 September 23). Vasculitis. National Heart Lung and Blood Institute. Available online at Accessed on 6/07/15.

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(© 2015). Symptoms of vasculitis. The Johns Hopkins Vasculitis Center Available online at Accessed on 6/07/15.

Gota, C. (2013 May, Reviewed). Overview of vasculitis. Merck Manual for Healthcare Professionals. Available online at Accessed on 6/07/15.

Mayo Clinic Staff (2014 October 8, Updated). Vasculitis. Available online at Accessed on 6/07/15.

(© 2015). Vasculitis. Central Manchester and Manchester Children's University Hospitals, Department of Immunology. Available online at Accessed on 6/07/15.

Mayo Clinic Staff. (2012 December 19). Wegener's granulomatosis. Mayo Clinic. Available online at Accessed on 6/10/15.

Hypersensitivity Vasculitis: Background, Pathophysiology, Epidemiology. Patel, M et al. Medscape. Available online at Accessed August 2015.

A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-vessel Vasculitis. Megan R. Goeser; Valerie Laniosz; David A. Wetter. Am J Clin Dermatol. 2014;15(4):299-306.

Sources Used in Previous Reviews

MedlinePlus.Vasculitis. Available online at Accessed February 2011.

National Heart Lung and Blood Institute. Vasculitis. Available online at Accessed February 2011.

The Johns Hopkins Vasculitis Center. Available online at Accessed February 2011. 

The Johns Hopkins Vasculitis Center. Symptoms of Vasculitis. Available online at Accessed February 2011.

Gota, C. (Updated 2008 May). Introduction: Vasculitis. Merck Manual for Healthcare Professionals [On-line information]. Available online at Accessed February 2011.

Mayo Clinic Staff (2009 October 10). Vasculitis [On-line information]. Available online at Accessed February 2011.

Central Manchester and Manchester Children's University Hospitals, Department of Immunology. Vasculitis. Available online at Accessed February 2011.