Vasculitis and Lupus: When Inflammation Targets the Blood Vessels
When Inflammation Targets the Blood Vessels
SLE causes damage throughout the body, including the bone marrow, the spleen, liver, kidneys, stomach, and intestines. It also can attack the blood vessels themselves. This is called vasculitis. You can read more about SLE-related changes to the bloodstream, such as inflammation-related anemia, here.
When blood vessels are attacked by lupus (and other autoimmune diseases like rheumatoid arthritis), inflammation and irritation cause the walls of the blood vessels to thicken, narrowing the blood vessels and restricting how much blood can flow. It can also close off the blood vessels completely, weaken the walls of the blood vessels, and cause bulges called aneurysms, which can become too weak, break, and bleed into the body.
Vasculitis and Lupus
Vasculitis is usually uncommon (1/100,000 people under 50). However, the risk increases with age and when a person has inflammatory diseases such as lupus. It can be caused by infections, smoking, and using illicit drugs. It can also be genetic, running in families. People with lupus find it a very common symptom and will generally develop vasculitis earlier in life than the general population.
The Circulatory System, Vasculitis, and Lupus
The circulatory system is the most vital series of organs in the body. The heart pumps the blood through the body; the lungs feed oxygen to the blood and help remove carbon dioxide. The kidneys filter out waste products from the blood and excess water to be released through the bladder as urine. When lupus causes a problem with the circulatory system, such as vasculitis, it can affect the whole body.
Blood vessels are small channels, like pipes. Blood is pumped by the heart and flows through these blood vessels throughout the body, delivering oxygen and nutrients to organs throughout the body and carrying away waste products produced by the cells. It then travels to the lungs, dumps the poisonous waste products like carbon dioxide, picks up more oxygen, and returns to the heart to flow through the body again.
In people with lupus or other autoimmune disorders, their antibodies mark the body’s own cells for destruction and bind to cells that make up the blood vessel walls. They are much more likely to do this if the blood vessels are already irritated or damaged, but either way, the body then attacks these marked cells. The blood vessels react, protecting themselves by thickening the walls and closing in on themselves, cutting off blood flow and leading to vasculitis.
If the flow of blood is too restricted, vital organs and tissues become starved of oxygen and nutrients and can’t work properly. In some cases, the restriction can be so severe that the organs can end up damaged. Poor blood flow can also increase the risk of clots forming, which can be especially damaging when it happens in the brain.
Symptoms of Vasculitis
SLE-caused vasculitis restricts blood flow and can harm any organs or tissues in the body as a result. The body also reacts to the choking effects of vasculitis by building more blood vessels so that more blood can arrive and make up for the lack of oxygen. Unfortunately, these new blood vessels can clog and clutter organs, making them less efficient.
Because of these wide-ranging effects, vasculitis has several key symptoms:
Headaches and Dizziness
Brain fog and other cognitive problems
Fatigue and reduced stamina
Fever
Visible spots, lumps, or sores
Pale, clammy skin
Rashes or blemishes on the skin
Poor appetite and weight loss
Cough, shortness of breath
Joint pain
Numbness, especially in the extremities (the further-out parts of the body such as the hands and feet)
The nervous system is especially sensitive to being cut off from oxygen, so sensory nerves, the brain, and organs such as the ears and eyes can be damaged. The lungs and heart are part of the circulatory system, so they can sometimes have the most severe symptoms. The digestive system (the stomach, intestines, and other organs) has trouble working together properly when their oxygen is limited. Vasculitis can weaken the walls of the blood vessels as well, which could allow them to break and cause an aneurysm—a form of internal bleeding that can potentially be life-threatening.
Inflammation and Vasculitis
In people with autoimmune disorders like lupus, inflammation doesn’t turn off. It’s always on the attack, and their immune system thinks that parts of their body are invaders, so it starts to damage parts of the body.
Inflammation is key to vasculitis, because in addition to individual organs and tissues being damaged, such as the heart, lungs, and the blood vessels themselves, inflammation also changes how the bloodstream behaves.
Tests like aiSLEⓇ (FRI) don’t detect vasculitis itself, but they measure immune system activity that signals rising inflammation. If the test results are extremely elevated, it may identify that there is an increased risk of a vasculitic lupus flare.
Cardiovascular Disease and Vasculitis - How are they Related?
Cardiovascular disease, including heart attacks and strokes, are the most common cause of death for people with lupus, with some studies saying that the rate is as high as 33%.
“Cardiovascular disease” is an umbrella term for any condition that affects the heart or blood vessels. Because vasculitis is inflammation of the blood vessels themselves, it can be considered a cardiovascular disease under this broad definition.
However, in lupus care, the term “cardiovascular disease” often refers to something different:
the increased risk of heart attacks, strokes, and early atherosclerosis caused by long-term inflammation.
Here’s how the two fit together:
Vasculitis and Atherosclerosis
Atherosclerosis is the formation of plaques, a buildup of substances that are naturally present in the bloodstream. These substances include cholesterol, fats, and other substances that the body needs to flow through the body, but in areas that don’t flow properly due to inflammation, they can end up “sticking” to the walls of blood vessels. As this sediment grows in arteries, the available space for the blood flow becomes constricted. As plaque grows, it encourages more cholesterol and fats to stick to it, continuing to grow.
Vasculitis, on the other hand, is the narrowing of blood vessels due to inflammation damaging the walls of blood vessels, causing them to thicken. Although atherosclerosis is not the same as vasculitis, narrow blood vessels can make plaques more likely to form. Both conditions restrict blood flow and thicken the walls of blood vessels, making the circulatory system less effective and potentially causing serious harm. Atherosclerosis and vasculitis require different medications but are both treatable with medications and diets.
Inflammation is important for both atherosclerosis and vasculitis, because it changes how platelets and other immune system-related molecules interact with blood vessels and the rest of the body. These changes can make both conditions more likely to happen and can worsen symptoms that are already there.
The Brain, Lupus, and Vasculitis
Vasculitis can affect brain function, or cognition. When it restricts blood and oxygen flow to the brain, it can cause or worsen brain fog and other neuropsychiatric symptoms. People with lupus also have a higher risk of developing a stroke, about 4-8 times higher according to studies. You can read more about strokes and lupus, here.
Treating Vasculitis and Lupus
Because vasculitis shares symptoms with other diseases, it's important for a medical team to figure out what is actually going on. A few conditions that doctors need to rule out include antiphospholipid syndrome (increased clotting), medication-related side effects that clench the blood vessel walls, and atherosclerosis. These can all be affected by lupus, but they require different treatment.
Tests for vasculitis include blood tests, which look for inflammation markers in the bloodstream. Imaging tests, such as x-rays, ultrasounds, CT scans, MRIs and PETs, are a way that medical professionals can look into the body and see the structure of muscles, blood vessels, and organs. Doctors might recommend a biopsy, too, where a sample is removed directly from the body and looked at under a microscope to see if there are signs of vasculitis.
When vasculitis is diagnosed in people with lupus, it’s a good idea to keep an eye on it, even when symptoms are mild. Not only can vasculitis become severe as other symptoms change, but even mild vasculitis can contribute to organ damage, and it’s best to catch that quickly.
Medications for vasculitis include anticoagulants, also known as blood thinners. Anticoagulants are used to treat and prevent strokes and other symptoms of vasculitis by making it more difficult for blood to clot. Anti-inflammatories and immunosuppressants such as glucocorticoids, methotrexate, and cyclophosphamide are often used to treat the underlying inflammation and lupus.
A Lupus Warrior’s Takeaway
Vasculitis can add another layer of challenge to living with lupus, but it’s treatable and manageable with the right care. Because it affects blood flow, symptoms can show up almost anywhere in the body—from skin and nerves to organs—so keep an eye out and bring up to your doctor. Vasculitis should be a priority for a lupus treatment team to manage.