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.