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Vasculitis isn’t one disease. Rather, it’s an umbrella term for a group of rare diseases that involve inflammation of the blood vessels.

If you have vasculitis, your blood vessels may stretch and get bigger. Vasculitis can also make your blood vessels narrow or close completely, leading to organ damage. In the worst cases, it can be life-threatening.

Vasculitis can occur suddenly or build up over weeks or months. It may affect one area of the body or the entire body. It occurs in both men and women equally and can happen at any age.

What Causes Vasculitis?

Doctors don’t always know what causes vasculitis. Sometimes it happens as a side effect from medication. It can also be the result of an allergy or another disease.

Triggers for vasculitis include:

• Autoimmune disorders.
• Infections such as hepatitis B or C.
• Cancer, such as lymphoma or leukemia.
• An allergic reaction.

Types of Vasculitis

There are about 20 types of vasculitis. Doctors classify them by the size and location of the affected blood vessels. Some of these types are:

Large vessel vasculitis

• Giant cell arteritis. This is an autoimmune disease that causes inflammation in the large arteries of the body, including the aorta, in adults older than age 50. The Vasculitis Foundation estimates about 228,000 Americans have this form of vasculitis.
• Takayasu’s arteritis. Inflammation of the walls of the aorta and its branches. It mostly occurs in younger women.

Medium vessel vasculitis

• Kawasaki disease. This form of vasculitis mostly strikes children. About 80% of people who have it are under age 5. It affects the mucous membranes, lymph nodes, blood vessel walls, and heart.
• Polyarteritis nodosa. This type of vasculitis happens most often in middle-aged men. It can affect the blood vessels of the skin, nervous system, kidneys, digestive system, and heart.

Small vessel vasculitis

• Primary angiitis of the central nervous system vasculitis. This is an inflammation of blood vessels in the brain or spine.
• Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). People who have this condition often suffer from asthma and sinus polyps. Inflammation of the blood vessels can also affect the nerves, heart, skin, and kidneys.
• Granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis). This is an inflammation in the small blood vessels of the lungs, kidneys, upper airways, skin, nerves, and sinuses.
• Microscopic polyangiitis. Inflammation of small blood vessels of the kidneys, lungs, nerves, skin, and joints. Common symptoms are high blood pressure and shortness of breath.

Vasculitis in blood vessels of various sizes

• Rheumatoid vasculitis. This is a complication of long-term rheumatoid arthritis. Inflammation spreads through the small and medium-sized blood vessels in the body. It can lead to complete blockage and cut off blood supply to an affected organ.
• Bechet’s disease. This form of vasculitis mostly affects people of Middle Eastern or East Asian descent. It can cause sores in the mouth, skin, and genitals, as well as joint and muscle pain.

Vasculitis Signs and Symptoms

Vasculitis symptoms can vary widely. You may have a general sense of being unwell, or feel unusually tired. You may lose your appetite.

Some people have mild symptoms, and others have severe symptoms. Vasculitis symptoms can resemble those of other diseases, so your doctor may want to rule out other problems first.

Vasculitis can cause the following symptoms in various organ systems.

Blood

Symptoms include:

• Elevated platelet count.
• Elevated white blood cell count.
• Low red blood cell count (anemia).
• Blood clots.

Brain

Symptoms include:

• Headache.
• Memory loss.
• Stroke.
• Vision changes.

Ear, nose, throat, and sinuses

Symptoms include:

• Change in voice.
• Ear pain.
• Hearing loss.
• Hoarseness.
• Inflammation and infection.
• Nasal congestion.
• Nose bleeds.
• Nose or mouth sores.

Eye

Symptoms include:

• Inflammation.
• Vision loss.
• Red eye.

Heart

Symptoms include:

• Angina.
• Heart attack.
• Chest pain.

Kidneys

Symptoms include:

• Blood in urine.
• Kidney failure.
• Protein in urine.

Lungs

Symptoms include:

• Abnormal chest x-ray or CT scan.
• Chest pain.
• Cough.
• Coughing up blood.
• Shortness of breath.

Nerves

Symptoms include:

• Numbness.
• Shooting pain.
• Tingling.
• Weakness in arms or legs.

Stomach and colon

Symptoms include:

• Abdominal pain or cramping.
• Bleeding from stomach or bowels.
• Perforated intestine.

Complications of Vasculitis

Vasculitis can be mild, but more severe cases can cause complications and even be life-threatening. Some of the complications of vasculitis include:

• Aneurysm. If the blood vessel stretches, it can bulge and risks bursting. If it bursts, it can cause dangerous bleeding inside the body.
• Stroke, vision changes, and memory loss. If vasculitis blocks blood flow to the brain, it can cause brain function problems.
• Heart attack. If vasculitis blocks blood flow to the heart, you can have a heart attack.
• Kidney failure. If vasculitis is in the blood vessels of the kidneys, it can lead to kidney problems.

Vasculitis Diagnosis

Because vasculitis isn’t just one disease, there are different ways of diagnosing it. You should always consult your doctor if you have vasculitis symptoms. Your doctor may refer you to a specialist for treatment, depending on your symptoms and medical history.

The earlier your doctor diagnoses vasculitis, the better your chances of getting it under control.

Depending on your symptoms, your doctor may order:

• Blood tests — Certain antibodies might indicate vasculitis.
• Urine tests — This allows your doctor to check for kidney damage.
• Imaging tests (x-rays, EKG, CT scans, MRI scans, and ultrasound) — This allows your doctor see if there is inflammation in the blood vessels.
• Angiography — This procedure involves a doctor injecting dye into the blood to show inflammation on imaging tests more clearly.
• Biopsy — This procedure involves a doctor takes tissue samples from an organ to look for signs of inflammation.

Vasculitis Treatment

Vasculitis treatment depends on what type of vasculitis you have. A mild case of vasculitis might go away on its own without treatment. The main goal of vasculitis treatment is to slow or stop the inflammation of the blood vessels.

Multidisciplinary care

Many different types of doctors may work together to coordinate your vasculitis care. Some of them are:

• Rheumatologists
• Nephrologists
• Pulmonologists
• Dermatologists
• Ophthalmologists
• Neurologists
• Otolaryngologists
• Cardiologists
• Vascular surgeons

Medications

Doctors often treat vasculitis with a strong drug such as a corticosteroid for a few months. The goal is to calm the inflammation and put the vasculitis in remission. Later, your doctor may prescribe a long-term milder drug to keep flare-ups at bay.

Medications may include:

• Corticosteroids (like prednisone) to reduce blood vessel inflammation.
• Immunosuppressive drugs, which can also reduce blood vessel inflammation, sometimes with fewer side effects than corticosteroids. Some are cyclophosphamide, rituximab, methotrexate, azathioprine, mycophenolate mofetil, leflunomide, tocilizumab, TNF inhibitors, and avacopan.

Plasmapheresis (plasma exchange)

Is used in severe cases to remove harmful antibodies from one’s
plasma with a procedure similar to dialysis.

Surgery

In severe cases, your doctor may recommend surgery to treat damage to blood vessels. A vascular bypass graft redirects blood flow around a blockage in a blood vessel. Your doctor may recommend transplant surgery to replace damaged/failed organ resulting from vasculitis.

Even if your vasculitis goes away, your doctor will want to see you for regular checkups. Sometimes the disease can flare up again. If you have any vasculitis symptoms, call your doctor right away.

Vasculitis Research at UPMC

While getting clinical care at the UPMC Vasculitis Center you might have the opportunity to participate in research and contribute to advancing our understanding and options for treatment for these rare diseases.