Ashton Kutcher on Monday revealed that he was “lucky to survive” vasculitis, a rare disorder that he claimed left him unable to “see, hear, or walk,” a couple of years ago. This is a group of rare autoimmune disorders that cause inflammation of blood vessels. The inflammation causes thickening or swelling of the vessel walls, affecting the smooth flow of blood in the body, in turn resulting in organ and tissue damage.

There are multiple types of vasculitis, depending on the site of the body and the type of blood vessels affected. It is diagnosed on the basis of symptoms, medical history, and laboratory tests. Vasculitis ranges from mild to severe, and although it may cause death in severe cases, it is largely treatable.

What is vasculitis?

Vasculitis is a term for a number of autoimmune disorders — or disorders in which the body’s immune system attacks healthy cells. Vasculitis may cause inflammation in the arteries, veins, or capillaries. Under usual circumstances, inflammation is the natural way in which the immune system responds to injuries or infections. It helps drive away invading germs and foreign pathogens. In vasculitis, however, inflammation occurs in healthy blood vessels — this disrupts the flow of blood from the heart to vital tissues and organs, and vice versa, eventually leading to damage to those organs and tissues.

The cause of vasculitis in a patient is often unknown. At times, patients’ genetic makeup may attract some kinds of vasculitis such as Takayasu arteritis or Wegener’s granumalotosis. In other instances, it could just result from the immune system incorrectly attacking blood vessels. The severity of vasculitis may range from a minor affliction to major damage to vital organs like the kidneys or the heart, or in the case of Kutcher, the eyes and ears and the brain.

There exist different types of vasculitis depending on the site and type of blood vessels that the immune system targets. There are vasculitis varieties that affect the lungs and respiratory system, which are different from varieties that attack the kidneys, which in turn are different from the types that attack the brain and nerves.

Who is at risk of vasculitis?

Anyone can get vasculitis, although different types of vasculitis may occur more frequently among different population groups based on their age, site of attack, sex, or a combination of these factors. It’s still unclear what causes it — although genetic factors could be one reason.

Based on age, for instance, one type of vasculitis called Churg-Strauss syndrome is known to cause asthma, symptoms of cold, and pain in muscles and joints, and occurs more frequently among adults between the age of 30 and 45. Another type of vasculitis called giant cell arteritis attacks the head and neck and usually occurs only in adults above the age of 50. The Kawasaki disease, characterized by rashes, swollen neck glands, and red eyes, occurs mainly in children under the age of 5.

Microscopic polyangiitis is another typethat particularly affects the lungs, kidneys, and nerves. There is also polyarteritis nodosa which is among the rarer type of vasculitis that specifically affects the arteries supplying blood to the gut, kidneys, and nerves. Behcet’s disease, which is known to usually cause ulcers in the mouth and genitals, is yet another form of the condition that occurs more commonly in populations in Japan, China, Greece, Turkey, and the Middle East than in people in the rest of the world.

There are also vasculitis types that are known to affect women more than men. For example, polymyalgia rheumatica, a vasculitis very similar to giant cell arteritis, attacks mainly women over the age of 50. Takayasu arteritis, on the other hand, mostly targets young women.

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What are the symptoms of vasculitis?

Symptoms of vasculitis vary depending on the type of vasculitis one gets afflicted with, but there are some common symptoms found across most varieties of vasculitis that can often manifest as “flare-ups.” These include fever, headache, fatigue, weight loss, and general body pain. Many vasculitis varieties are also known to cause rashes and lesions in the skin. Churg-Strauss syndrome can cause asthma, cold-like symptoms caused by allergy, and numbness by affecting nerves. Giant cell artiritis, on the other hand, can cause double vision or vision loss, and may cause jaw pain to the patient while eating.

Yet another variant, called Wegener’s granumalotosis is known to cause night sweats, nosebleeds, and inflammation of sinuses. Rob, a patient from the United States, documents his experience of living with Wegener’s granumalotosis to Vasculitis Foundation, recounting that “I would run a high fever, joints would swell, and soon I had no energy.”

Jeanne and John, whose fourteen-month-old daughter Jessica contracted Takayasu artiritis, recall that, “Jessica developed unexplainable high fevers, ear infections, and respiratory problems including asthma. She had tubes put in her ears on her second birthday… Finally, at the age of four…they found the narrowing and inflammation in her arteries…”

Can vasculitis be treated or cured?

Vasculitis is treatable. Most treatment strategies focus on managing the inflammation and controlling the underlying conditions which may have triggered the vasculitis. Treatment also involves suppressing the immune system. Medication usually involves synthetically produced corticosteroid drugs, or drugs that recreate hormones that are usually found in the adrenal cortex of the brain. A common corticosteroid prescribed for vasculitis is prednisone. However, corticosteroids may often produce severe side-effects, especially if taken for a long period of time.

At times, other medications, such as methorexate, tocilizumab, or rituximab, are prescribed alongside corticosteroids to taper their dosages quickly. Specific medications may also be prescribed depending on the severity, the organs affected, and other medical conditions of a vasculitis patient.

Vasculitis may at times cause an unusual bulge or ballooning — called an aneurysm — of an affected blood vessel. Surgical removal of the bulge may be necessary for averting the risk of the blood vessel rupturing. Sometimes, surgery may also be required to treat blocked arteries in order to restore the blood flow in a site affected by vasculitis.