WHAT IS VASCULITIS?

Recently the actor Ashton Kutcher informed the public that he had recently been diagnosed with vasculitis which affected his ability to see, hear and walk. But what exactly is this condition?

Vasculitis is a condition whereby the blood vessels are inflamed. This compromises the blood vessel structure and can, as a result, lead to organ and tissue damage. Read further to learn about the causes, who is at risk of vasculitis, the symptoms, and more.

Causes of vasculitis

Vasculitis is believed to likely be an autoimmune disease in which the body’s own immune system erroneously attacks normal body cells. Once activated, immune system cells and chemicals appear to attack the walls of the blood vessels.

Vasculitis can be classified as either primary or secondary. Secondary vasculitis is when the illness is caused by a known factor while primary has no known cause. In the case of secondary vasculitis, a couple of triggers exist.

These factors that have been shown to activate vasculitis are listed below.

• Toxins
• Infections
• Inflammatory conditions
• Cancer

Who is at risk for vasculitis?

Anyone can be affected by vasculitis.

Some factors that increase your risk of vasculitis include the following:

• Genetics: if someone in your family has been diagnosed with the disease, then it is more likely you will get ill. This means that there are likely genes that can be inherited which can impact your odds of developing the disease.
• Cocaine use: cocaine is a drug that has many harmful effects on multiple organ systems, and it increases your chance of developing vasculitis.
• Tobacco use: smokers and people who chew tobacco are at a bigger risk of vasculitis.
• Hepatitis infections: a person with either hepatitis B or C virus is more likely to develop vasculitis.
• Sex: whether you are a male or female determines your risk of developing a particular type of vasculitis. In fact, males are more prone to developing the particular form of vasculitis known as Buerger’s disease; females are more susceptible to giant cell arteritis, a different type of vasculitis.

Symptoms of vasculitis

The signs of vasculitis can be somewhat vague and a person may assume they have some viral infection. Vasculitis symptoms include the following:

• Fever
• Head pain
• Losing weight
• Generalized pain in the body

Signs of vasculitis will vary in accordance with the part of your body that is affected by the inflammation.

• Lungs: if your lungs are affected then you may have some difficulty breathing or you may even experience hemoptysis (coughing up blood).
• Digestive organs: pain and discomfort in the gut after eating; there may also be blood in the stool.
• Extremities: having a numb feeling in the extremities, weakness, or swelling of the feet and hands.
• Ears and eyes: ears may ring and eyes may redden and itch; blindness is also possible in certain types of vasculitis.

It is always best to see your doctor and not assume you simply have the flu or some other viral infection, since there can be dangerous complications with vasculitis.

Complications of vasculitis

There are several complications that can happen if you have vasculitis. These are described below.

• Blood clots: clots can block arteries in the heart and brain leading to heart attacks and strokes.
• Aneurysms: this is when one part of a blood vessel enlarges.
• Vision loss: the loss of vision can mean permanent blindness.
• Damaged organs: inflamed blood vessels in the organs affect the function of the organs since it compromises the flow of oxygen and nutrients to the organ.

Types of vasculitis

There are several different types of vasculitis that are classified based on the part of the body that is afflicted as well as the size of the blood vessels involved.

A list with a description of each type of vasculitis is provided below.

• Takayasu’s arteritis: affects the central nervous system.
• Behçet disease: inflamed mucosal membranes resulting in ulcers forming in these membranes.
• Buerger's disease: inflammation of blood vessels of the legs and arms.
• Giant cell arteritis: affects carotid arteries and the aorta; these are all large arteries in the body.
• Kawasaki disease: affects the kidneys.
• Cutaneous vasculitis: impacts the skin.
• Polyarteritis nodosa: adversely affects the digestive system.
• Rheumatoid Vasculitis: this is when the inflammation of joints from rheumatoid arthritis spreads to nearby blood vessels.
• Cryoglobulinemic vasculitis: usually caused by hepatitis and affecting the joints most often.
• Henoch–Schönlein purpura (HSP): a common vasculitis of childhood that causes kidney inflammation (nephritis).
• Eosinophilic granulomatosis with polyangiitis (EGPA): also known as Churg Strauss; it is quite a rare form of vasculitis which affects sinuses, the lungs, heart, and nervous system.
• Granulomatosis with polyangiitis: also called Wegener’s disease; affects the lungs, ears, throat, nasal cavities, and kidneys.

Vasculitis and pregnancy

Vasculitis can pose a threat to a developing fetus. The woman’s health may also be jeopardized if her vasculitis is severe. Careful management is required in the case of women who have vasculitis and then fall pregnant.

Vasculitis diagnosis

It is important that a diagnosis of vasculitis is made as soon as possible so that treatment of the condition can begin. Diagnosis is possible by means of the following tests:

• Blood tests: looking for inflammatory markers such as C-reactive protein, AST, ALT, creatine, and BUN levels.
• Laboratory tests: often these tests involve searching for certain antibodies such as cryoglobulins and antibodies for hepatitis. The laboratory tests help determine the type of vasculitis and its cause.

Treatment of vasculitis

Since vasculitis is an inflammatory condition, the treatment option usually involves using medications such as corticosteroids such as rituximab and possibly also immunosuppressants such as methotrexate. These medications help decrease the immune system response that is responsible for the damage that is caused to the blood vessels.

Treatment options also depend on why the person has vasculitis. If the underlying cause is known, then treatment of that condition is helpful. However, as mentioned before, not all causes of vasculitis are known.

Conclusion

Vasculitis is an inflammatory condition in which there is inflammation of the blood vessels. The illness is often debilitating and can be life-threatening. Rapid and correct diagnosis is essential in the management of this illness to avoid dangerous complications that could lead to death.

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Sources:

• Clowse, M. E., Richeson, R. L., Pieper, C., Merkel, P. A., & Vasculitis Clinical Research Consortium. (2013). Pregnancy outcomes among patients with vasculitis. Arthritis care & research, 65(8), 1370-1374. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4366137/#:~:text
• Davin, J. C., & Coppo, R. (2014). Henoch–Schönlein purpura nephritis in children. Nature Reviews Nephrology, 10(10), 563-573. https://www.nature.com/articles/nrneph.2014.126
• Ferri, C., & Mascia, M. T. (2006). Cryoglobulinemic vasculitis. Current opinion in rheumatology, 18(1), 54-63. https://journals.lww.com/co-rheumatology/Abstract/2006/01000/Cryoglobulinemic_vasculitis.10.aspx
• Jennette, J. C., & Falk, R. J. (1997). Small-vessel vasculitis. New England Journal of Medicine, 337(21), 1512-1523. https://www.nejm.org/doi/full/10.1056/NEJM199711203372106
• Villa-Forte, A. (2022). Overview of vasculitis. https://www.msdmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/overview-of-vasculitis

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