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Vasculitis, also known as angiitis and arteritis, is an inflammation of the blood vessels (arteries, veins & capillaries). Arteries carry blood from your heart to your body's organs. Veins carry blood from your organs and limbs back to your heart. Capillaries connect the small arteries and veins.

  • “Inflammation” refers to the body’s response to injury, including injury to the blood vessels. Inflammation may involve pain, redness, warmth, swelling, and loss of function in the affected tissues.

It causes changes in the blood vessel walls, including weakening, stretching, thickening, narrowing, or scarring. The blood vessel can either increases in size or become narrow--even to the point of entirely closing. These changes can restrict blood flow, resulting in organ and tissue damage.

  • The vessel may weaken so much that there may be stretching and a bulge (aneurysm) develops.
  • In extreme conditions, if the vessel wall becomes too weak, it may rupture and bleed, possibly causing death, a very rare event.
  • If a blood vessel becomes inflamed and narrowed, the blood supply to the area of the body it serves may be partially or completely blocked. If alternate blood vessels (called collateral blood vessels) are not available in sufficient quantity to carry the blood to such sites, the tissue supplied by the affected vessels will die. Because vasculitis can occur in any part of the body, any tissue or organ can be affected.

There are many types of vasculitis, and most of them are rare. Vasculitis might affect just one organ, or several. The condition can be short term (acute) or long lasting (chronic) and can range from mild to life-threatening.

Vasculitis can affect people of all ages, though some types are more common among certain groups.

Some of the many forms of vasculitis may be restricted to particular organs. Examples include vasculitis that affects only the skin, eye, brain, or certain internal organs. There are also types of vasculitis that may affect many organ systems at the same time. Some of these generalized forms may be quite mild and may not require treatment. Others may be severe, affecting critical organs.

Early detection and treatment of severe vasculitis can prevent permanent damage. Detection of vasculitis most often requires blood tests, biopsy of affected tissue or angiography. Depending on the type some may improve without treatment. Some types require medications to control the inflammation and prevent flare-ups.

Vasculitic diseases are inflammatory health problems that often need treatment with medications such as glucocorticoids. Patients also may be prescribed other medicines that suppress the immune system. These can help severe disease or let patients take lower doses of glucocorticoids.

Types include, but not limited to the following:

Symptoms

The signs and symptoms of vasculitis vary greatly. They're often related to decreased blood flow throughout the body. The disruption in blood flow caused by inflammation can damage the body's organs. Signs and symptoms depend on which organs have been damaged and the extent of the damage.

General signs and symptoms common to most vasculitis types

See also: Johns Hopkins Vasculitis Center - Symptoms of Vasculitis (lists sigs/symptoms by location in the body with photos)

  • General symptoms: Fever, weight loss
  • Skin: Palpable purpura, livedo reticularis
  • Muscles and joints: Myalgia or myositis, arthralgia or arthritis
  • Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss
  • Heart and arteries: Myocardial infarction, hypertension, gangrene
  • Respiratory tract: Nose bleeds, bloody cough, lung infiltrates
  • GI tract: Abdominal pain, bloody stool, perforations
  • Kidneys: Glomerulonephritis

Signs and symptoms for Specific Types of vasculitis

Other signs and symptoms are related only to certain types of vasculitis. The symptoms can develop early and rapidly or in later stages of the disease.

  • Behcet's (beh-CHETS) disease. This condition causes inflammation of your arteries and veins. Signs and symptoms include mouth and genital ulcers, eye inflammation, and acne-like skin lesions.
  • Buerger's disease. This condition causes inflammation and clots in the blood vessels of your hands and feet, resulting in pain and ulcers in these areas. Rarely, Buerger's disease can affect blood vessels in the abdomen, brain and heart. It is also called thromboangiitis (throm-boe-an-jee-I-tis) obliterans.
  • Churg-Strauss syndrome (Eosinophilic granulomatosis with polyangiitis). This condition is very rare. It mainly affects the lungs, skin, kidneys, heart and nerves in your limbs. Signs and symptoms vary greatly and include asthma, skin changes, nerve pain and nasal allergies.
  • Cryoglobulinemia. This condition results from abnormal proteins in the blood. Signs and symptoms include rash, joint pain, weakness, and numbness or tingling.
  • Giant cell arteritis. This condition is an inflammation of the arteries in your head, especially at the temples. Giant cell arteritis can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. It is also called temporal arteritis.
  • Granulomatosis with polyangiitis. This condition causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Signs and symptoms include nasal stuffiness, sinus infections, nosebleeds and possibly coughing up blood. But most people don't have noticeable symptoms until the damage is more advanced.
  • Henoch-Schonlein purpura (IgA vasculitis). This condition is more common in children than in adults, and causes inflammation of the smallest blood vessels (capillaries) of your skin, joints, bowel and kidneys. Signs and symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your buttocks or lower legs.
  • Hypersensitivity vasculitis. Sometimes called allergic vasculitis, the primary sign of this condition is red spots on your skin, usually on your lower legs. It can be triggered by an infection or an adverse reaction to medicine.
  • Kawasaki disease. This condition most often affects children younger than age 5. Signs and symptoms include fever, rash and redness of the eyes. It is also called mucocutaneous lymph node syndrome.
  • Microscopic polyangiitis. This form of vasculitis affects small blood vessels, usually those in the kidneys, lungs or nerves. You may develop abdominal pain and a rash, fever, muscle pain and weight loss. If the lungs are affected, you may cough up blood.
  • Polyarteritis nodosa. This form of vasculitis usually affects the kidneys, the digestive tract, the nerves and the skin. Signs and symptoms include a rash, general malaise, weight loss, muscle and joint pain, abdominal pain after eating, high blood pressure, muscle pain and weakness, and kidney problems.
  • Takayasu's (tah-kah-YAH-sooz) arteritis. This form of vasculitis affects the larger arteries in the body, including the aorta. Signs and symptoms include joint pain, loss of pulse, high blood pressure, night sweats, fever, general malaise, appetite loss, headaches and visual changes.

Causes

The exact cause of vasculitis isn't fully understood. Some types are related to a person's genetic makeup. Others result from the immune system attacking blood vessel cells by mistake. Possible triggers for this immune system reaction include:

  • Infections, such as hepatitis B and hepatitis C
  • Blood cancers
  • Immune system diseases, such as rheumatoid arthritis, lupus and scleroderma
  • Reactions to certain drugs

Blood vessels affected by vasculitis may bleed or become inflamed. Inflammation can cause the layers of the blood vessel wall to thicken. This narrows the blood vessels, reducing the amount of blood — and therefore oxygen and vital nutrients — that reaches your body's tissues and organs.

Risk factors

Vasculitis can occur in any sex or race or at any age.

  • Smoking
  • Having chronic hepatitis B or C infections
  • Having some types of autoimmune conditions, including rheumatoid arthritis, scleroderma or lupus

Complications

Vasculitis complications depend on the type and severity of your condition. Or they may be related to side effects of the prescription medications you use to treat the condition.

  • Complications of vasculitis include:
    • Organ damage. Some types of vasculitis can be severe, causing damage to major organs.
    • Blood clots and aneurysms. A blood clot may form in a blood vessel, obstructing blood flow. Rarely, vasculitis will cause a blood vessel to weaken and bulge, forming an aneurysm (AN-yoo-riz-um).
    • Vision loss or blindness. This is a possible complication of untreated giant cell arteritis.
    • Infections. These include serious and life-threatening conditions, such as pneumonia and blood infection (sepsis).

Prognosis

The outcome of vasculitis is hard to predict. It will depend on the type of vasculitis, which organs are affected, and the severity of the condition.

If vasculitis is diagnosed early and responds well to treatment, it may go away or go into remission. "Remission" means the condition isn't active, but it can come back, or "flare," at any time.

Resources & Additional Reading:

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