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Vasculitis

Vasculitis refers to a group of conditions that cause inflammation in the blood vessels.

At the UPMC Division of Vascular Surgery, we collaborate with experts from many disciplines to diagnose and treat vasculitis. Specialists we work with include joint and muscle, infectious disease, skin, lung, kidney, nervous system, heart, eye, and urinary tract experts.

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What Is Vasculitis?

Vasculitis is a group of conditions that causes inflammation in the blood vessels.

It can be:

  • Systemic, affecting multiple systems in the body.
  • Isolated to a specific blood vessel group.

Vasculitis is complex and can range from mild to life threatening.

It may happen once in a person’s life and never recur. In some cases, vasculitis can recur after successful treatment.

Some people need lifelong treatment for vasculitis.

Vasculitis causes and risk factors

Vasculitis happens when your immune system attacks your blood vessels by mistake.

While doctors don't know exactly why this happens, some risk factors can include:

  • Autoimmune disorders like lupus, rheumatoid arthritis, and scleroderma.
  • Medicines.
  • Infections like hepatitis B, hepatitis C, or viral illness.
  • Blood cancers.

Vasculitis complications

Vasculitis can damage blood vessels, causing them to become thick, weak, narrow, or scarred. This can limit blood flow, keeping your organs and tissues from getting the oxygen and nutrients they need.

Complications from vasculitis can include:

  • Organ damage.
  • Blood clots.
  • Aneurysm, or a bulge or weak spot in a blood vessel.
  • Vision loss or blindness.
  • Infections like pneumonia and sepsis (blood infection).
  • Limb loss.

Types of Vasculitis

There are many types of vasculitis.

Behçet's disease

  • Causes ulcers in the mouth and genitals, skin lesions, and eye inflammation.
  • Usually affects people age 20-40. Men are more likely to have it than women.

Buerger’s disease (tormboangiits obliterans)

  • Causes swelling and blood clots in the small and medium-sized arteries in the hands and feet. This leads to pain, discoloration, and skin ulcers.
  • Linked to tobacco use.

Cogan’s syndrome

  • Mainly affects the aorta and aortic valve in people with systemic vasculitis.
  • Can cause eye inflammation, hearing changes, and deafness.

Cryoglobulinemia vasculitis

  • Can cause symptoms like rash, joint pain, weakness, and tingling or numbness.
  • Usually associated with hepatitis C.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

  • Very rare type of vasculitis.
  • Can affect the lungs, skin, kidneys, nervous system, and heart with varied symptoms.

Giant cell arteritis

  • Causes inflammation in the arteries of the scalp and head, but can affect any artery.
  • Common symptoms include headache, jaw pain, and vision problems.
  • Mostly affects people age 50 and older.

Hypersensitivity vasculitis

  • Can cause red spots on the skin, mainly in the lower legs.
  • Usually associated with a reaction to medicine or an infection.

IgA vasculitis (Henoch-Schonlein purpura)

  • Can cause rash, abdominal pain, swollen and painful joints, and blood in urine.
  • Most common in children between 2 and 11 years old.
  • Often follows a respiratory, throat, or gastrointestinal infection.

Kawasaki disease (mucocutaneous lymph node syndrome)

  • Rare condition that affects children.
  • Causes fever, rash, and eye inflammation.

Microscopic polyangiitis

  • Usually affects the kidneys and lungs.
  • Can cause fever, weight loss, and muscle aches.

Polyarteritis nodosa

  • Can cause fever, general malaise, weight loss, muscle and joint aches, anemia, rash and other skin changes, and stomach pain after eating.
  • Often associated with hepatitis B.

Polymyalgia rheumatica

  • Affects the shoulders and hips, causing pain and stiffness.
  • Often associated with giant cell arteritis.

Takayasu’s arteritis

  • Causes inflammation in the aorta and its branches, and in the arteries of the arms, brain, and abdominal organs.
  • Can cause stroke in severe cases.

Vasculitis Symptoms and Diagnosis

Symptoms of vasculitis

Symptoms of vasculitis can vary greatly, but often include:

  • Fever
  • Aches and pains, including headaches
  • Rash
  • Fatigue
  • Shortness of breath or cough
  • Numbness or pain in a hand or foot

Depending on what organs or body systems it affects, other vasculitis symptoms can include:

  • Mouth ulcers
  • Itching
  • Stomach pain
  • Sinus or inner ear infections
  • Eye itching or burning
  • Trouble thinking clearly
  • Stroke-like symptoms, including muscle weakness

Diagnosing vasculitis

To diagnose vasculitis, your UPMC vascular surgeon will ask about your medical history and symptoms.

He or she will perform a physical exam and may use the following tests to confirm your diagnosis:

  • Blood tests to check for inflammation
  • A biopsy of a blood vessel to confirm inflammation
  • Imaging tests like an x-ray, CT scan, or MRI

Vasculitis Treatment

The main goal of vasculitis treatment is to reduce and control the inflammation in your blood vessels.

Treatment options include:

  • Medicines like steroids and immunosuppressant drugs.
  • Blood thinners.
  • Plasmapheresis, a process that filters certain proteins from your blood.
  • Angioplasty, a catheter-based procedure that uses a balloon to open blocked blood vessels.
  • Bypass surgery to restore blood flow.

In some cases, your UPMC vascular surgeon may successfully treat your vasculitis, but then it returns.

Some people need ongoing treatment for the rest of their lives.

Living with vasculitis

Vasculitis can require short-term or lifelong treatment, depending on your condition and symptoms.

Some people who need ongoing treatment can experience:

  • Side effects from medication
  • Fear
  • Anxiety
  • Depression
  • Stress

Vasculitis management

To help manage your vasculitis:

  • Stay on your treatment plan, and talk with your doctor about any medication side effects. Your doctor may be able to adjust the dose to help reduce side effects.
  • Quit or avoid smoking.
  • Choose healthy foods to help control your risk factors for vascular disease, like your blood pressure, cholesterol, and blood sugar levels.
  • Get and stay active. Regular physical activity can help lower your risk for high blood pressure and diabetes. It can also help you manage stress.
  • Ask for help. Talk with your friends and family. Consider speaking with a counselor or joining a support group for people living with vasculitis.