Vasculitis

DEFINITION

Vasculitis is an inflammation of your blood vessels. It causes changes in the walls of blood vessels, including thickening, weakening, narrowing and scarring. These changes restrict blood flow, resulting in organ and tissue damage.

There are many types of vasculitis, and most of them are rare. Vasculitis might affect just one organ, such as your skin, or it may involve several. The condition can be short term (acute) or long lasting (chronic).

Vasculitis can affect anyone, though some types are more common among certain groups. Depending on the type you have, you may improve without treatment. Or you will need medications to control the inflammation and prevent flare-ups.

Vasculitis is also known as angiitis and arteritis.

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.

SYMPTOMS

The signs and symptoms of vasculitis vary greatly and are often related to decreased blood flow throughout the body.

General signs and symptoms common to most vasculitis types

General signs and symptoms of vasculitis include:

• Fever
• Headache
• Fatigue
• Weight loss
• General aches and pains
• Night sweats
• Rash
• Nerve problems, such as numbness or weakness
• Loss of a pulse in a limb

Signs and symptoms for some types of vasculitis

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

• Behcet’s (beh-CHET’S) syndrome. This condition causes inflammation of your arteries and veins. It generally appears in your 20s and 30s. Signs and symptoms include mouth and genital ulcers, eye inflammation, and acne-like lesions on your skin.
• Buerger’s disease. This condition causes inflammation and clots in the blood vessels of your hands and feet. It can cause pain in your hands, arms, feet and legs, and ulcers on your fingers and toes. This disorder is linked with cigarette smoking. It is also called thromboangiitis (throm-boe-an-jee-I-tis) obliterans.
• Cryoglobulinemia. This condition results from abnormal proteins in the blood. It is often associated with hepatitis C infections. Signs and symptoms include rash, joint pain, weakness, and numbness or tingling.
• Eosinophilic granulomatosis with polyangiitis (also called Churg-Strauss syndrome). This condition is very rare. It mainly affects the kidneys, lungs and nerves in your limbs. Symptoms vary greatly and include asthma, nerve pain and sinus changes.
• Giant cell arteritis. This condition is an inflammation of the arteries in your head, especially at the temples. It usually occurs in people over age 50. Giant cell arteritis can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. It is also called temporal arteritis.

  Giant cell arteritis is linked to polymyalgia rheumatica, which causes pain and stiffness in muscles of the neck, shoulders, hips and thighs.

• Granulomatosis with polyangiitis (also called Wegener’s granulomatosis). This condition causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Signs and symptoms include nasal stuffiness, sinus infections and nosebleeds. The affected tissues develop lumps called granulomas. If the lungs are affected, you may cough up blood. The kidneys are often affected. But most people don’t have noticeable symptoms until the damage is more advanced.
• Henoch-Schonlein purpura. This condition is an 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. Henoch-Schonlein usually affects children, but it can occur at any age.
• Hypersensitivity 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 eye inflammation. It is also called mucocutaneous lymph node syndrome.
• Microscopic polyangiitis. This form of vasculitis affects small blood vessels, usually those in the kidneys and lungs. You may develop abdominal pain and a rash. 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. It is often associated with hepatitis B infections. Signs and symptoms include a rash, muscle and joint pain, abdominal pain, high blood pressure, and kidney problems.
• Takayasu’s (tah-kah-YAH-sooz) arteritis. This form of vasculitis affects the larger arteries in the body, including the aorta. It typically occurs in young women. Signs and symptoms include a feeling of numbness or cold in the limbs, loss of pulse, high blood pressure, headaches, and visual changes.

When to see a doctor

Make an appointment with your doctor if you have any signs or symptoms that worry you. Some types of vasculitis can get worse quickly, so early diagnosis is key to getting effective treatment.