Vasculitis What Is Vasculitis?

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Vasculitis describes a group of rare conditions, also called angiitis, that damage blood vessels by causing inflammation, or swelling. Vasculitis can affect any size and type of blood vessel (arteries and veins), anywhere in the body.

Vasculitis is called primary when the cause cannot be identified. It is called secondary when something else causes it to occur. Potential triggers of this inflammation include infections, trauma, some drugs, and autoimmune disorders (diseases that cause your immune system to abnormally react against normal cells and tissues in your body). The inflamed lining (walls) of blood vessels may restrict or completely block blood flow, or it may cause formation of blood clots. In rare cases an inflamed vessel wall may balloon, forming an aneurysm with thin walls at risk of tearing.

With vasculitis, you may experience general symptoms, such as fever, weight loss, tiredness, pain, and rash. You may have other symptoms depending on the part of the body that is affected and the severity of vasculitis. If you are diagnosed with vasculitis, medicine can help improve your symptoms and help you avoid flares and complications. With treatment, vasculitis can go into remission, which is a period of time when the disease is not active.

There are many types of vasculitis. Each type affects different parts of the body and can lead to different symptoms. Some of the more common types are listed below.

• Anti-glomerular basement membrane disease affects blood vessels in the lungs and kidneys.
• Behçet’s disease may affect many areas of the body, including the mouth, eyes, skin and genitals.
• Buerger’s disease, also known as thromboangiitis obliterans, usually affects blood flow to the arms and legs.
• Central nervous system vasculitis, also called primary angiitis, affects the blood vessels (small arteries and veins) in the central nervous system: the brain, spinal cord, and the protective membranes that cover both. There are two types of central nervous system vasculitis.
  • Primary central nervous system vasculitis means the disease is located only in the central nervous system and not linked with other diseases.
  • Secondary central nervous system vasculitis means the disease exists along with other autoimmune diseases such as lupus or rheumatoid arthritis, other forms of vasculitis, or infection.
• Cogan’s syndrome is an autoimmune disorder that mainly affects the eyes and inner ears along with large blood vessels.
• Cryoglobulinemic vasculitis affects the small blood vessels and is often linked with hepatitis C. It prevents proper blood flow and causes pain and damage to the skin, joints, peripheral nerves, kidneys, and liver.
• Eosinophilic granulomatosis with polyangiitis, also known as Churg-Strauss syndrome, often affects the respiratory tract, gastrointestinal tract, skin, heart and nervous system.
• Giant cell arteritis, also known as temporal arteritis, mostly affects the aorta and its major branches. The condition often affects the temporal artery in the head and may lower blood flow to the optic nerve, causing vision loss.
• Granulomatosis with polyangiitis usually affects the nose and throat area, lungs, and kidneys.
• Hypersensitivity vasculitis affects the skin. This condition also is known as allergic vasculitis, cutaneous vasculitis, or leukocytoclastic vasculitis.
• Hypocomplementemic urticarial vasculitis is linked with inflammation in the small blood vessels and low blood levels of certain proteins, which affects the body’s ability to develop defenses against infection.
• Immunoglobulin A (IgA) vasculitis, formerly known as Henoch-Schönlein purpura, is one of the most common types of vasculitis in children but can also affect adults. It develops when IgA, which is a type of antibodies that usually helps defend the body against infections, builds up in blood vessels of the skin, joints, intestines, and kidneys. This causes inflammation and leakage of blood.
• Kawasaki disease is a rare childhood disease that affects blood vessels throughout the body, commonly the coronary arteries. Kawasaki disease is also known as mucocutaneous lymph node syndrome.
• Microscopic polyangiitis affects small to medium-sized blood vessels, often including those in the kidneys and lungs.
• Polyarteritis nodosa causes damage, including aneurysms, most often to medium-sized arteries. This type of vasculitis may cause muscle and joint pain, kidney failure, high blood pressure, or symptoms such as abdominal pain, nausea, and vomiting.
• Retinal vasculitis affects small blood vessels in the eye and can cause problems with vision.
• Takayasu arteritis most often affects the aorta and its branches. The condition can also affect medium-sized arteries. It most frequently affects adolescent girls and young women and is more common in Asia.