What is vasculitis?

Vasculitis, also known as angiitis or arteritis, is a general term for a group of about 20 different blood vessel inflammation disorders. These disorders trigger changes to the walls of the blood vessels, including thickening, weakening, narrowing, and eventually scarring. All forms of vasculitis are similar in that they involve blood vessel inflammation, but the affected organs or system can be different. The condition can be acute and be resolved or it can become chronic.

In vasculitis, the immune system, which normally protects the body, attacks the blood vessels causing inflammation. Inflammation can involve pain, redness, warmth, swelling, or loss of function in the affected tissue or part of the organ. The disruption in blood flow caused by inflammation can damage the body's organs.

Vasculitis can also be part of several rheumatic diseases, especially lupus, rheumatoid arthritis, and Sjögren's syndrome.

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

EGPA is a rare form of vasculitis, formerly known as the Churg-Strauss Syndrome. EGPA is characterized by asthma, eosinophilia (an excessive number of eosinophils or white blood cells in the blood), fever, and vasculitis of different organ systems. The presence of granulomas (masses of inflamed tissue) in addition to the increased eosinophils are the distinguishing aspects that separate EGPA from other forms of vasculitis. Granulomas can be particularly frightening because on X-rays, they can look like cancerous masses. Granulomas eventually calcify and have the same density as bone. EGPA typically affects middle-aged people with a history of new-onset or newly-worsened asthma. Men and women have an equal likelihood of getting the disease.

Granulomatosis with Polyangiitis (GPA)

Formerly called Wegener’s Granulomatosus or Wegener’s disease, GPA is a form of vasculitis that most frequently affects the upper respiratory tract (sinuses, nose, ears, and trachea), lungs, and kidneys. GPA is characterized by granulomas and necrosis (irreversible tissue death due to lack of blood flow). The granulomatosis may stay in one organ system for a few months or years before spreading to other organs. It is slightly more common in males and typically occurs in middle age but can affect people at any age.

Microscopic Polyangiitis (MPA)

Although this form of vasculitis occurs rarely, MPA is distinguished from GPA and other similar forms of vasculitis because it affects small- to medium-sized blood vessels of the kidneys, nervous system (particularly the peripheral nerves, as opposed to the brain or spinal cord), skin, and lungs. MPA is such a rare disease that there is little information available about long-term consequences but on average, five years after diagnosis, more than 80 percent of people with MPA have survived and are trying to achieve remission. MPA affects all ages and men and women equally.

What causes vasculitis?

The cause of vasculitis is not fully understood. It is rarely seen in two family members, ruling out genetics as a major risk factor. Possible risk factors that can trigger an 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

What are the symptoms of vasculitis?

The symptoms of vasculitis vary depending on which blood vessels are inflamed, causing decreased blood flow and which organ systems are affected. However, general signs and symptoms that most people with vasculitis experience include:

  • Fever
  • Fatigue
  • Weight loss and/or loss of appetite
  • Muscle and joint pain or general achiness with headache
  • Night sweats
  • Rash
  • Nerve problems, such as numbness or weakness

Symptoms of EGPA vary greatly but include asthma, nerve pain, and sinus changes. The earliest symptoms of EGPA are nasal polyps and allergic rhinitis. After an individual has EGPA for some time, an excessive number of eosinophils (a type of white blood cell) build up in the blood or in tissues. If left untreated, later symptoms of EGPA are severe tingling, numbness, shooting pains, and severe muscle wasting with eventual loss of strength in the hands or feet.

Symptoms of GPA include nasal stuffiness, sinus infections, and nosebleeds. Fever, fatigue, and weight loss are also common. Affected tissues can develop granulomas which look like lumps. If you have GPA in your lungs, you may cough up blood. When GPA is in your kidneys, there are no specific symptoms until the disease is advanced and damage has occurred. Because of the disease’s quiet onset, it is important to communicate with your rheumatologist if you experience any of the general vasculitis symptoms as soon as possible.

A range of symptoms are associated with MPA, most commonly a general feeling of ill health, fatigue, abdominal pain, fever, and loss of appetite and/or weight. Other symptoms depend on which organ systems are involved.

  • If MPA affects the lungs, you may have shortness of breath or cough up blood.
  • MPA affecting the nerves may cause an abnormal sensation followed by numbness or loss of strength.
  • Kidney disease caused by MPA does not have specific symptoms until kidney damage occurs.
  • Arthritis (inflammation of the joints accompanied by swelling) can also be a symptom of MPA. Joint pain tends to move between joints. For example, a patient might feel it in the right shoulder one day and in the left wrist the next.

How is vasculitis diagnosed?

It is difficult to diagnose any type of vasculitis without a thorough physical exam and a detailed description of your medical history. There are also laboratory tests and procedures that are useful in diagnosing the specific form of vasculitis including:

  • Blood tests look for signs of inflammation, such as a high level of C-reactive protein, anemia, and white blood cell count.
  • Urine tests show whether your urine contains red blood cells or has too much protein. Urine tests can show evidence of kidney inflammation before any specific kidney symptoms are experienced.
  • Chest X-rays and computerized tomography (CT) are different imaging techniques that look for granulomas in the chest and identify GPA. X-rays are less expensive and involve less exposure to radiation but do not have the accuracy of a CT scan for observing granulomas in their early stages.
  • Angiography, or an X-ray of your blood vessels, is performed using a flexible catheter that is inserted into a large artery or vein. A dye is injected through the catheter and into the vein or artery while an X-ray is taken to show how the dye flows through the arteries or veins.
  • Biopsy looks at a small tissue samples that are surgically taken from the affected area of your body to determine if the mass has signs of vasculitis. Biopsies are invasive and not always decisive in their diagnosis. Lungs and kidneys are the most common organs to undergo biopsy to diagnose vasculitis.
  • Asthma is the distinctive feature of EGPA though only a small minority of patients with asthma have vasculitis. But when combined with other symptoms, diagnostic tests for EGPA will be conducted.
  • EGPA-specific tests include eosinophil count, tests for carpal tunnel nerve-type numbness, X-rays for indications of lung inflammation, blood tests for antibodies common in people with EGPA, and tests for sinus abnormalities.

How is vasculitis treated?

Vasculitis can be a temporary or life-long problem. Treatment for all forms of vasculitis focuses on controlling inflammation (getting to remission) and preventing a relapse of symptoms. Achieving remission and preventing relapse relies on specific drug combinations; these combinations depend on which organs are involved and what complications you are experiencing.

In addition to prescription medications, there are lifestyle modifications that can help manage your vasculitis and help you stay in remission longer.

  • Eat a healthy diet. Some medications have side effects that can be managed or treated with a healthy diet. For example, corticosteroids may require you to have more vitamin D and calcium. Other drugs can cause thinning bones, high blood pressure, and diabetes that a diet emphasizing fresh fruits and vegetables, whole grains, low-fat dairy products, and lean meats and fish can help manage.
  • Get vaccinated. Some medications compromise your immune system; staying up-to-date on vaccinations, such as for flu and pneumonia, can help prevent these illnesses.
  • Exercise. Weight-bearing exercise, such as walking, running, and other forms of aerobic exercise and strength training can help prevent the bone loss, high blood pressure, and diabetes that are side effects of some medications such as corticosteroids.
  • Know when to ask for help. Maintain a strong support system, including seeking out vasculitis support groups, so that you can get help if symptoms impact your ability to be independent or compromise your emotional health.
  • Communicate with your doctor. Understand your treatment plan and tell your doctor when you have new symptoms.

Complications of vasculitis

Complications of vasculitis depend on the type and severity of disease but can include organ damage, blood clots or aneurysms, vision loss, and infections such as pneumonia and sepsis.