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Vasculitis: Treatment For Autoimmune Symptoms
Medically reviewed by Stella Bard, MD
Vasculitis is a medical condition involving swollen or inflamed blood vessels that can affect different parts of the body. It can be caused by viruses such as hepatitis B or C, other infections, side effects of medications, cancer, or autoimmune diseases such as systemic lupus erythematosus (SLE). When the blood vessels become inflamed, it inhibits blood flow and can lead to complications, including organ damage.
Learn about vasculitis, its causes and risk factors, types, treatment options, and more.
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Autoimmune Effects of VasculitisAn autoimmune reaction happens when the immune system attacks healthy cells in the body. This can lead to inflammation in various organs and regions of the body, sometimes in the entire body. When the blood vessels that carry blood throughout the body—including arteries, veins, and capillaries—become inflamed, it can prevent blood flow. Since blood flow is required for the body to function properly, this can lead to various complications.
Causes and Risk FactorsVasculitis can have many different causes, and sometimes the cause is not known.
Some possibilities include:
Some people are more likely to experience vasculitis than others, and risk factors vary for the different types of vasculitis. Some types of vasculitis are more common among women, while other types are more common among men.
Similarly, some types are more likely to happen later in life, while others, such as Kawasaki disease and IgA (immunoglobulin A) vasculitis, are more likely to affect children. Healthy lifestyle choices can help to prevent vasculitis from happening.
Symptoms: How Do You Know You Have Vasculitis?The symptoms of vasculitis vary depending on the type and the part of the body affected. There can be a rash, which may look like hives, or a spotty red or purple rash that most commonly affects the legs and lower regions of the body, but can also show up on the upper areas of the body.
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Other symptoms of vasculitis may include:
Consult a healthcare provider about these or other symptoms. The effects of vasculitis can be severe and life-threatening.
Emergency Vasculitis SymptomsEmergency symptoms of vasculitis include:
Seek immediate medical attention if you or someone you know experiences these symptoms.
Vasculitis Classification and TypesThere are three primary classifications of vasculitis—large, medium, and small vessel vasculitis—based on the size of the blood vessels they affect. Other types of vasculitis, including Behcet's syndrome and relapsing polychondritis, can affect various sizes of blood vessels.
Large Vessel VasculitisLarge vessel vasculitis includes:
Giant cell arteritis (GCA) may affect the eyes, joints, and aorta. It typically occurs in adults over 50 and can cause headaches, jaw pain, flu-like symptoms, fatigue, and fever. GCA can also lead to eye problems and vision loss.
Takayasu's arteritis affects large and medium arteries and artery branches, with widespread inflammation and damage. It is more common among young women and involves symptoms such as loss of appetite, weight loss, fever, and a general unwell feeling.
Aortitis affects the primary blood vessel to the heart (aorta). It can have different causes, including systemic lupus erythematosus (SLE), so no specific category of people is most susceptible. Symptoms may include decreased appetite, fatigue, fever, headache, pain in the chest, and weight loss.
Medium vessel vasculitis includes:
Kawasaki disease (KD) or Kawasaki syndrome involves inflammation of the blood vessels that carry blood to the heart. It is more common among young children, typically before age five, and can cause eye redness, fever, mouth irritation, and heart damage.
Polyarteritis nodosa affects the blood vessels of various body areas, such as the digestive tract, heart, joints, nervous system, and skin. It is more common among men of middle age, and symptoms may include breathing challenges, fatigue, pain in the abdomen or joints, sores on the skin, weakness or loss of feeling in the hands or feet, or weight loss.
Small vessel vasculitis includes:
Granulomatosis with polyangiitis (GPA), or Wegener's granulomatosis, involves inflammation of the blood vessels of areas of the body, such as kidneys, lungs, and sinuses. It is more common among men of middle age. It may lead to symptoms such as breathing challenges, cough, fatigue, fever, kidney issues, nasal congestion, painful joints, runny nose, or nosebleeds.
Microscopic polyangiitis involves inflammation of the blood vessels and can affect the lungs, kidneys, nerves, and skin. It is more common among men of middle age, and symptoms may include blood in the urine, breathing challenges, a cough that can consist of blood, pain in joints or muscles, and rash on the skin.
It can be challenging to get a vasculitis diagnosis, especially when the symptoms develop slowly, sometimes over years, which can happen with Takayasu's arteritis. The diagnostic process may differ depending on the type, but it generally includes a physical exam by a healthcare provider, a medical history and symptom evaluation, and testing. The testing may include blood tests, imaging such as x-rays or ultrasound, biopsy, and urinalysis.
Treatment to Manage VasculitisThe treatment of vasculitis depends on the type, symptoms, severity, and part of the body affected. If you have vasculitis, a healthcare provider may prescribe medications to reduce inflammation and open the blood vessels, restoring blood flow.
These medications may include over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs), such as Advil or Motrin (ibuprofen) or Aleve (naproxen sodium), or prescription anti-inflammatory medications for more severe cases. If an underlying autoimmune condition causes your vasculitis, a provider may prescribe medications to suppress the immune system to stop the autoimmune response.
Sometimes, a combination of medications is used to minimize the side effects of more powerful drugs.
Long-Term ConsiderationsOne of the most significant long-term considerations for vasculitis is its impact on the body, especially if left untreated. Vital organs can be damaged, sometimes severe enough to cause organ failure when they do not receive enough blood. With treatment, the outlook for vasculitis is good, with a high survival rate and improved quality of life as treatments can decrease symptoms.
Resources and SupportThere are many resources available and options for support for anyone experiencing vasculitis. The Vasculitis Foundation is a great place to start with learning resources and opportunities to connect with support on their website, including virtual support meetings.
Additionally, there are more specific resources and support options for different types of vasculitis, with some local options available. If you or someone you know is experiencing vasculitis, talk to a healthcare provider about resources and support.
SummaryVasculitis is a medical condition that involves restricted blood flow due to inflamed blood vessels. An autoimmune response causes some types of vasculitis. Other causes may include cancer, infections, and smoking. The symptoms vary, but people with this condition may experience a rash, fatigue, pain, and more.
There are different types, and reaching a diagnosis may be challenging or take time. Treatment can help to reduce the inflammation, open the blood vessels to restore blood flow, alleviate symptoms, and improve your quality of life.
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What To Know About Lupus And Vasculitis
Vasculitis is a common complication of active lupus that affects your blood vessels. Symptoms depend on which blood vessels are involved and can range from skin issues and headaches to seizures and strokes.
Vasculitis is a condition that involves inflammation that can damage your blood vessels. Depending on the type, vasculitis can affect various blood vessels throughout your body. While vasculitis itself is rare, it's not an uncommon complication of active lupus.
If you or a loved one has lupus and you're concerned about the possible development of vasculitis, consider talking with a doctor about the following possible signs and symptoms, as well as your treatment options.
Vasculitis is inflammation of the blood vessels. Many things can trigger vasculitis. In the case of lupus, vasculitis develops when your immune system attacks your own blood vessels.
Doctors classify vasculitis as either "primary" or "secondary." Primary vasculitis is idiopathic, meaning there's no underlying cause. Secondary vasculitis means something has caused the condition. Severe disease activity in autoimmune diseases like lupus is linked to an increased chance of vasculitis.
When vasculitis develops as a complication of lupus, it's known as lupus vasculitis. While it can affect any area of the body, it typically affects the skin.
Other possible causes of vasculitis in people with lupus include:
General symptoms of vasculitis can include:
However, depending on the exact type of vasculitis you have, you might experience more signs and symptoms affecting a particular body part or system. These include your:
Diagnosing vasculitis in lupus involves reviewing your medical history and the signs and symptoms you're experiencing. As with lupus, no single test can determine whether you have vasculitis.
To start, a doctor may order:
It's also possible for lupus vasculitis to present with a positive antineutrophil cytoplasmic antibody blood test result.
There's no single treatment for lupus vasculitis. Instead, a doctor will focus on treating the affected areas of your body. Common treatments include steroids or other immune system-suppressing drugs that help prevent your immune system from attacking blood vessels.
Treating vasculitis is essential for alleviating related symptoms but can also help reduce your chance of complications.
If you have lupus, it's also important to keep following your treatment plan as recommended by a doctor. While some lupus medications may increase your risk of vasculitis, you shouldn't stop taking them without first talking with a healthcare professional.
When blood vessel inflammation occurs in vasculitis, the vessel walls thicken. Over time, this can cause your blood vessels to narrow and weaken. Subsequently, this can increase your chance of related complications.
Possible complications of lupus vasculitis generally include:
Organ damage may develop because blood cannot flow to them as efficiently.
Your outlook with lupus vasculitis depends on which part of your body is affected. Vasculitis involving internal organs, like your lungs or digestive system, may have a poorer outlook than other areas of the body.
For example, mesenteric vasculitis is an extremely rare complication of lupus that affects the blood vessels in your gastrointestinal tract. About half of people who develop this complication die without prompt diagnosis and treatment, according to a 2021 review.
While treatment can help you manage symptoms, vasculitis is a chronic condition that requires long-term treatment. Without treatment, vasculitis may worsen your outlook with lupus, depending on the extent of inflammation and subsequent damage.
Consider discussing the following common questions about lupus vasculitis with a doctor.
How common is lupus vasculitis?Lupus vasculitis is relatively common, affecting about 11–36% of people with lupus, though some studies put the figure as high as 50%. However, the severity of the condition varies greatly, with some people experiencing mild vasculitis and others developing life threatening complications.
What autoimmune diseases cause vasculitis?While vasculitis can develop on its own, it may also be due to other autoimmune diseases. In addition to lupus, these include Sjögren disease, scleroderma, and rheumatoid arthritis.
What is the life expectancy of people with lupus vasculitis?The exact life expectancy of people with lupus vasculitis isn't known. With treatment, most people with lupus have average lifespans. However, developing vasculitis can affect this, particularly if there is damage to major organs.
Vasculitis, or inflammation of your blood vessels, is a common complication of active lupus. While the symptoms of vasculitis are wide-ranging and depend on which blood vessels are affected, it's important to report any unusual symptoms to your doctor.
Like lupus, vasculitis is treatable even though there isn't a cure. Prompt treatment may help prevent further complications associated with thickened and weakened blood vessels.
A Medical Student Was Isolated By An Autoimmune Disorder. Poetry Was A Way Out
When Leena Danawala's poetry was published in JAMA last month, the medical journal became just about the only place online to publicly showcase her art.
Danawala, a 34-year-old rheumatologist in the Chicago area, had been writing poetry for over a decade but only posted about it occasionally on her Instagram. During her two-year rheumatology fellowship at Ohio State University, she suddenly felt the urge to share.
"There were a lot of people who had the same disease I had. And when I talked to them about it, they were so relieved to hear that there was somebody who got through medical school and still came out the other side and is still doing OK," she told STAT.
Danawala's condition, a form of small vessel vasculitis called granulomatosis with polyangiitis, was diagnosed when she was in her early 20s. Through poetry, she can see the wider view: the illness "made me into like a new person, forged a new life."
STAT spoke to Danawala about her JAMA poem, "chronicity," the process of getting medical answers, and forming a new identity. This interview has been edited for clarity and brevity.
When did you write "chronicity" and what inspired it?
I wrote this like a year ago, while I was thinking about my journey. It felt like a very long hallway that's never going to end, because they couldn't diagnose me at first. For two years I was having these symptoms and they didn't really know what it was, and none of the treatments were working. And that's also why my parents turned to all alternative pathways.
I think doing that made them feel better, so that's why I did it. A lot of times they'd be like, "Well, let's go see this priest, and you do this puja," which is like a ceremony, like a prayer to god. I did a ton of those. Or some priest would read my horoscope, the Indian version of it. It's hard for me to really believe in that, to be honest. But there's also no harm in fulfilling your parents' requests. So that's why I put some of those things in there.
You wrote, "being ill is being alone." Can you talk about that feeling?
I don't know if it's necessarily all Indians, but definitely a lot of people from India don't really tell anyone besides close family about illnesses. And it might be something found in other cultures as well. I think part of it is, especially when you're a woman, marriageability. And then part of it is just you don't want someone else knowing the family's business or judging you for an illness. Or it's a fear of losing opportunities.
So when I first took my time off from medical school, I didn't even tell any of my friends why I was taking time off. A lot of my friends were very confused, and I lost touch with a lot of them. I really only have, like, two friends left from medical school because of that.
And at that time, too, I think it was just hard to find other people with similar symptoms as me.
Granulomatosis with polyangiitis causes inflamed blood vessels, including in the respiratory tract, ears, and internal organs. It is sometimes shortened to GPA. The condition was known as Wegener's granulomatosis (named after the pathologist who detailed the disease) until the early 2000s. But professional societies later agreed to rename the condition after researchers uncovered Friedrich Wegener's past as a high-ranking member of the Nazi Party. Your condition is an invisible illness, too. The only thing that was visible was I had uveitis. My left eye was constantly, completely red — very noticeably red. So that was the only thing that was visible besides me not looking like I was feeling good, like I had the flu or something. It was pretty isolating in that sense. That's when I picked up poetry again, mainly because that was the only outlet. Poetry became a little bit like journaling for me. You didn't want to take leave from medical school. What caused you to make that decision? I basically went to class, went home and went to sleep. To maintain my grades in medical school, I cut out a lot of social activities. I just stopped hanging out with people because I didn't have the energy. There came a time when I had to take a board exam after two years of medical school. I was studying for that and I just couldn't do it — you should be studying like eight hours a day for 4 to 6 weeks. And that determines what residency you can get into. I decided, because I couldn't do it, I didn't want to ruin my chances of my future career. Initially, when I took the time off, I thought it was only going to be three months. It ended up being almost two and a half years because my doctors couldn't really figure out what was going on. I went through treatment after treatment and none of it was working. later, i meet a priest who tells me part of me is a dead soul who cannot rest. Another says it is the planets and the way they set. — Leena Danawala, "chronicity" (JAMA Poetry and Medicine) How did you get a diagnosis? We were sick of it after a year of going from doctor to doctor and no one really giving us any answers, so we asked to go to the Mayo Clinic. They were the ones to diagnose me. They're the ones that did all the biopsies and did further imaging and retested some of the bloodwork. There are certain antibodies that are positive in this disease, and so those antibodies were retested and they were positive. They were already hearing about the new medication, so they were like, "We should just start you on it." Once I started on rituximab, it was kind of life-changing. So many things improved pretty quickly after that. Rituximab, marketed as Rituxan by Biogen and Genentech (among other brand names), is a monoclonal antibody treatment for autoimmune conditions, including GPA. Rituximab has been a blockbuster drug for at least five years, and sales of biosimilars have continued to increase. Rituximab may be combined with anti-inflammatory steroids. Broadly, the approach is to suppress the immune system to minimize organ damage. Drugs like rituximab, which is also used in oncology and delivered by infusion, attack and kill certain immune cells, called CD20+ B cells. Immunosuppressant drugs make people more susceptible to infections and viruses. I know I said your poetry was difficult to Google, but I did find out that in 2013, you won first place in a poetry contest hosted by a public library. And it was in that time frame of when you were getting a diagnosis. Being at home all day was kind of depressing. And so I would just go to the library and read or draw or write, stay there for a few hours and come home. I went through all the stages of grief during that time. There's just a lot of loss. The hardest part of getting a diagnosis is a loss of your sense of self — who you were as a person is no longer the same. And you have to be OK with building a new person. Who I was before was very athletic, I was involved in a gazillion things at once, obviously had a lot of hobbies, a lot of social obligations. I had to cut down a lot of those things, and it made me very frustrated and also really angry that I couldn't do the same things, angry at the limitations that I now had. It felt very much like a little box I'd put myself in. I had to re-identify who I was, and then how I could reintegrate some of the things that made me me, not at the cost of my health. The final line in "chronicity" says, "perhaps someday god will tell me i am fine." It made me think of an experience many people with chronic illness share: the difficulty of being in an in-between space — something is off and maybe you don't know exactly what, and you might sometimes feel broken but you don't want to be treated as if you're broken. Can you walk me through that final line? I think it is what you were mentioning. I say I'm in remission right now because generally my condition is well controlled. But there's good days and bad days. There's days where I thought I was going to be OK, and then by the end of the day I'm completely unable to do anything. So even though I'm technically better, I don't think I'm necessarily at the 100% that a normal person my age would be at. I'm always, constantly trying to find ways to be fine. It's very much a balance between pretending you're OK and then letting yourself not be OK. When I wrote that line, it just seemed to fit. That's what I think when I do anything religious in relation to my disease process, which is that at some point maybe the good will be greater than the bad, or I won't have to take medication anymore, or something like that. And then I won't have to do all the prayers. "Living With" explores the contours of life with chronic illness, from the prelude to diagnosis to new patterns of living, to wrestling with big questions about illness and health. Want to share your story of living with a chronic illness? Email [email protected]. STAT's coverage of chronic health issues is supported by a grant from Bloomberg Philanthropies. Our financial supporters are not involved in any decisions about our journalism.
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