Treatments
Your Treatment Plan
Treatment recommendations for vasculitis are based on the results of research studies and knowledge gained through clinical experience. However, they are just one tool to use as you and your healthcare provider build your treatment plan. Developing a treatment plan that is right for you requires careful discussion between you and your healthcare provider, guidance from research studies and treatment guidelines, and the clinical knowledge, experience and expertise of the members of your treatment team.
While different forms of vasculitis may share similar symptoms and treatment recommendations, each disease is distinct and each person with vasculitis is unique. It is important to remember that what works for one individual may not work for you. The Vasculitis Foundation provides information on vasculitis treatments as a resource to help promote shared decision making between you and your medical team.
Beginning Treatment
- Tell your healthcare provider if you have any pre-existing conditions, especially problems with your heart, kidneys, or liver.
- Tell your healthcare provider if you are pregnant or considering pregnancy or if you are nursing. Some vasculitis treatments can impact fertility in both men and women. There may also be a risk of birth defects or fetal harm associated with some vasculitis treatments. Learn more about vasculitis and reproductive health.
- Tell your healthcare provider about all medications and supplements you are taking including herbs. Medications and supplements can interact with each other in ways that can be dangerous or that can decrease how well a medication works.
- Serious allergic reactions to medications are a rare, but possible risk. Immediately notify your healthcare provider if you experience trouble breathing; notice swelling of your tongue, lips, throat, or face; feel faint, or have moderate or severe abdominal pain or vomiting.
- Many vasculitis treatments suppress your immune system. This means you will not be able to fight off infections as effectively. You will need to take extra precautions to help protect yourself from illness and infection while taking immunosuppressant treatments. Learn more about protecting youreself against illness and infection.
- It is important to have one medical provider who takes the lead in your treatment. Vasculitis patients often choose their rheumatologist for this role. You and other members of your treatment team should always check with the team lead before adding any new treatments or medications. You should also discuss any upcoming dental and medical procedures with your team lead. Learn more about building your treatment team.
- Ask your doctor about how to take your medication.
- How often?
- Is there a specific time of day? Glucocorticoids (steroids) are often taken in the morning for example.
- Are there any foods or beverages you should avoid? Alcohol needs to be avoided when taking some medications, others shouldn’t be taken with grapefruit juice.
- Should you take it on an empty stomach or with a meal?
Infusions are administered at a controlled pace through an IV. It can take 2-4 hours to receive an infusion. Infusions are given in an infusion center or hospital where you are carefully monitored.
Vasculitis medications or treatments that may be given by infusion include: abatacept, intravenous immunoglobulins, rituximab, and tocilizumab.
Infusion Tips
We went to the experts, people living with vasculitis, to get their best tips for preparing for an infusion.
- Hydrate Unless you have been told to restrict fluids, drink plenty of water the day before, during, and after your infusion.
- Nutrition is Important Too Eat well before and after your appointment. Bring snacks for during your infusion.
- Comfort is Key Wear comfy clothes, dress in layers (some people find they get hot during an infusion while others feel cold), and bring a cozy blanket.
- Communicate Be sure to share with your nurse if anythings feels off during the infusion. You know your body better than anyone else. Consider keeping a record of how you feel after infusions.
- Make Time Fly Bring a book, craft, or other project. Download podcasts, movies, and music in advance. Pack your headphones and a long charging cable.
- Nap Enough said.
- Treat Yourself with Kindness Give yourself some extra love and pampering. Be gentle with yourself. Have someone drive you and pick you up. Rest as much as you can. Get some fresh air. Ask for support.
- Mentally Reframe Meditate, think positive thoughts, consider this some well-deserved “me time.”
Remember these words of encouragement from others who “Get It.” – You’ve got this. Good luck. You’ll do great.
Injections are administered via a needle or an injection pen (autoinjector). Injections may be given by your medical provider, or you or a family member may be asked to administer your injections.
Vasculitis medications that may be given by injection include: abatacept, adalimumab, benralizumab, cyclophosphamide, etanercept, infliximab, mavrilimumab, mepolizumab, methotrexate, omalizumab, rituximab, sarilumab, and tocilizumab.
Tips for Giving Injections at Home
- Make sure you, or the person who will be giving the injection, is comfortable using the needle or the autoinjector before leaving the hospital or your healthcare provider’s office. Practice, ask questions, and practice some more.
- Ask your healthcare provider where on your body the injection should be given and whether or not you need to rotate the site of the injection.
- Thoroughly wash your hands before giving the injection.
- Always use an alcohol swab to clean the skin at the injection site before giving the injection.
- If possible, use a medical grade sharps container to dispose of needles and autoinjectors. You can substitute a thick, strong plastic bottle such as one that would be used to hold laundry detergent or fabric softener for the sharps container if necessary. The plastic should be thick enough that a needle won’t be able to penetrate it.
- Always handle needles and autoinjectors with care. Do not attempt to disassemble needles or autoinjectors. Do not attempt to remove batteries from autoinjectors.
- After giving the injection, do not try to recap the needle or remove it from the syringe. Promptly dispose of the needle by placing it in the appropriate plastic container.
- Do not overfill the sharps container. You risk sticking yourself if you try to jam a needle or autoinjector into a container that is too full.
- When the sharps container reaches the full mark (when it is about three-quarters of the way full) dispose of it properly. Ask your healthcare provider or pharmacist where the container should be taken for disposal. Sharps containers should not be thrown out with your household trash.
Johns Hopkins has a series of instructional videos on the proper technique for injecting different medications. The links to these videos can be found at https://www.hopkinsarthritis.org/patient-corner/how-to-give-a-subcutaneous-injection/
Vasculitis Treatments
The treatments discussed on this page are used for the treatment of vasculitis in the United States of America. Your access to these treatments may vary depending on where you live.
FDA Approved
Drugs receive FDA approval when the manufacturer demonstrates that its drug is “safe and effective” for the intended use, and that it can “manufacture the product to federal quality standards.” If the FDA grants an approval, it means that the agency has “determined that the benefits of the product outweigh the risks for the intended use.” Drugs that are FDA approved for one form of vasculitis, may occasionally be used off-label to treat other forms of vasculitis.
FDA approved to treat oral ulcers in Behçet’s syndrome
Apremilast is a phosphodiesterase 4 (PDE4) inhibitor. Apremilast aims to control inflammation by reducing the activity of an enzyme (protein that speeds up a reaction) called PDE4 inside the cells.
Given orally.
Possible Serious Side Effects
Significant weight loss
You and your healthcare provider will need to decide if you should continue to take apremilast if significant weight loss occurs.
Depression
Tell your healthcare provider if you have any feelings of depression or if you have suicidal thoughts.
Severe diarrhea, nausea and vomiting.
Notify your healthcare provider if you develop abdominal pain, diarrhea, nausea and vomiting.
FDA approved for the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Learn more about Tavneos® at Tavneos® Connect
Avacopan belongs to a class of medications called complement inhibitors. It works to modulate the response of your immune system by blocking the complement C5a receptor. It is considered an adjunctive treatment, meaning it is used in combination with the standard treatment. It may reduce your exposure to glucocorticoids such as prednisone by helping you to achieve and maintain remission with a lower cumulative (total amount over the course of the treatment) dose of glucocorticoids.
Given orally twice a day.
Possible Serious Side Effects
Serious Infection
Avacopan suppresses your immune system which makes it harder for your body to fight infection. The most common serious infections seen with avacopan were pneumonia and urinary tract infections. You and your healthcare provider will monitor for signs and symptoms of infection.
Hepatitis B virus (HBV) reactivation
If you have had HBV or are a carrier of HBV, taking avacopan could cause the virus to become an active infection again. If you notice worsening tiredness or yellowing of your skin or the white part of your eyes tell your healthcare provider immediately.
Liver injury
If your liver is impaired you may not be able to take avacopan. Your healthcare provider will order blood tests to test your liver function before and during treatment.
FDA approved for the treatment of Kawasaki disease.
What is IVIG? Intravenous immunoglobulin is an intravenous (iv) infusion given through a vein in your arm. It consists of immunoglobulins (antibodies) gathered from the plasma (liquid content of the blood) of thousands of healthy donors.
How long does it take? When used as a treatment for Kawasaki disease the infusion is typically given one time and takes several hours to complete.
Possible Side Effects
- Chills
- Headache
- Fever
- Nausea
- Rash
- Muscle Aches
Rare Side Effects
- Inflammation of the lining of the brain (aseptic meningitis).
- Severe allergic reaction sometimes occurs in patients who have certain forms of IgA deficiency
Tell your medical team
- if you have difficulty breathing, chest tightness or wheezing
- if you develop a rash or fever
- if you have severe and persistent headaches with neck stiffness, nausea and vomiting
FDA approved for the treatment of EGPA.
Mepolizumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Mepolizumab works by reducing the number of eosinophils, a type of white blood cell, in your blood. Reducing the number of eosinophils in your blood may help reduce inflammation.
Given by injection.
Possible Serious Side Effects
Shingles infection
You may need to be vaccinated for shingles before taking mepolizumab.
Serious allergic reaction
Contact your healthcare provider immediately if you have signs of a serious allergic reaction such as swelling of your face, mouth, or tongue; breathing problems, lightheadedness, or hives.
FDA approved for the treatment of granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA). May be used off-label to treat aortitis, cryoglobulinemic vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), IgA vasculitis, and rheumatoid vasculitis.
Rituximab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Biologics target certain parts of the immune system to control inflammation. Rituximab works by blocking certain cells and proteins of the immune system that are causing inflammation.
Biosimilars are similar to, but not exact copies of, biologic medications. Ruxience® and Truxima® are biosimilars of rituximab. The American Cancer Society’s Understanding Biologic and Biosimilar Drugs is a great resource if you want to learn more about biologics and biosimilars.
Given as an intravenous infusion. The effects begin about six weeks after the infusion. Full effects can usually be felt by the third month and usually lasts for 6 months.
Possible Serious Side Effects
Serious Infection
Rituximab suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Serious allergic reaction
You will be closely monitored during the infusion for signs of an allergic reaction. Before your infusion begins, you may be given medications to help prevent an allergic reaction. Tell your infusion nurse immediately if you experience signs of allergic reaction such as pounding or irregular heartbeat, shortness of breath, wheezing, or swelling of lips, tongue, or throat. Monitor yourself for signs of an allergic reaction after you leave the infusion center and contact your healthcare team immediately with any concerns.
Hepatitis B virus (HBV) reactivation
If you have had HBV or are a carrier of HBV, taking rituximab could cause the virus to become an active infection again.
Fetal harm
Use an effective form of birth control to prevent pregnancy during your treatment with rituximab and for 12 months after your final dose.
FDA approved for the treatment of polymyalgia rheumatica (PMR)
Sarilumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Biologics target certain parts of the immune system to control inflammation. Sarilumab stops inflammation by targeting a molecule in your immune system called interleukin 6 receptor (IL-6R).
Given as a subcutaneous injection.
Possible Serious Side Effects
Serious Infection
You and your healthcare provider will monitor for signs and symptoms of infection. Your doctor will also monitor your neutrophil count through a blood test. Neutrophils are a type of white blood cell that fights infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
High Cholesterol (hyperlipidemia)
You should have a blood test to measure your cholesterol (fasting lipid profile) before beginning treatment, 4-8 weeks into treatment, and then approximately every 6 months while taking Sarilumab.
Rarely, tears in the stomach or intestines
Inform your healthcare provider if you have a history of diverticulitis. Notify your healthcare provider immediately if you develop abdominal pain or have bloody bowel movements.
Liver injury
If your liver is impaired you may not be able to take sarilumab. Your healthcare provider will order blood tests to test your liver function before and during treatment.
FDA approved for the treatment of giant cell arteritis (GCA). May be used off-label to treat Takayasu arteritis (TAK).
Tocilizumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Biologics target certain parts of the immune system to control inflammation. Tocilizumab targets interleukin 6 (IL-6) a part of your immune system that promotes inflammation, which is known to be involved in giant cell arteritis (GCA). It works by blocking signals from IL-6 to your immune system. In clinical studies, tocilizumab—used in combination with tapering doses of glucocorticoids (steroids)—helped patients achieve sustained disease remission while significantly reducing their exposure to steroids.
Small studies of tocilizumab have shown good results in controlling inflammation and reducing the need for glucocorticoids in the treatment of Takayasu arteritis (TAK), but more research is needed.
Given as a subcutaneous injection or intravenous infusion.
Possible Serious Side Effects
High Cholesterol (hyperlipidemia)
You should have a blood test to measure your cholesterol (fasting lipid profile) before beginning treatment, 4-8 weeks into treatment, and then approximately every 6 months while taking tocilizumab.
Serious Infection
You and your healthcare provider will monitor for signs and symptoms of infection. Your doctor will also monitor your neutrophil count through a blood test. Neutrophils are a type of white blood cell that fights infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Liver injury
If your liver is impaired you may not be able to take tocilizumab. Your healthcare provider will order blood tests to test your liver function before and during treatment.
Off-Label
Many medications used to treat vasculitis are prescribed off-label. This means that while the medication has been approved by the Food and Drug Administration (FDA) to treat other diseases or medical conditions, it has not been specifically approved for the treatment of vasculitis.
Once the FDA approves a medication, healthcare providers may prescribe it for an unapproved use when they judge it is medically appropriate for their patient. This often occurs when there is not an approved medication to treat a specific disease or when your healthcare provider has not been able to successfully treat your disease despite trying all of the approved medications. Since there are few FDA approved medications for vasculitis, your healthcare provider may need to prescribe an off-label treatment.
You can learn more about off-label medications HERE.
May occasionally be used off-label to treat Giant cell arteritis (GCA) when there is a contraindication for Tocilizumab. It may also be used off-label in granulomatosis with polyangiitis (GPA), in certain specific situations when other medications cannot be used.
Abatacept is a biologic medication. Biologic medications are complex proteins derived from living organisms. Biologics target certain parts of the immune system to control inflammation. Abatacept works to lesson inflammation by attaching to the surface of inflammatory cells and blocking communication between these cells.
Given by subcutaneous injection or intravenous infusion.
Possible Serious Side Effects
Serious Infection
Abatacept suppresses your immune system which makes it harder for your body to fight infection You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Fetal Harm
Use an effective form of birth control. Tell your healthcare provider if you are pregnant or become pregnant while taking abatacept.
May be used off-label to treat Behçet’s syndrome.
Adalimumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. It also belongs to a class of medications called tumor necrosis factor (TNF) inhibitors. TNF is a protein in your body that causes inflammation. TNF inhibitors are drugs that block TNF and reduce inflammation
Given by subcutaneous injection.
Possible Serious Side Effects
Serious Infection
Adalimumab suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Rarely, lymphoma (cancer that begins the cells that fight infections) in children, teenagers, and young adults.
This was more common when adalimumab was being taken in conjunction with azathioprine for the treatment of Crohn’s disease or ulcerative colitis. Tell your health care provider if your child develops stomach pain; fever; unexplained weight loss; swollen glands in the neck, underarms, or groin; or easy bruising or bleeding.
May be used off-label to treat many forms of vasculitis.
Azathioprine belongs to a class of medications called disease-modifying anti-rheumatic drugs (DMARDs). Azathioprine works to reduce inflammation by suppressing the immune system.
TPMT testing: Your healthcare provider may order a blood test called TPMT activity level before treatment with azathioprine. Thiopurine methyltransferase (TPMT) is an enzyme that helps metabolize and clear azathioprine from your system. If you have lower levels of TPMT, you may be at a higher risk for medication toxicity.
Given orally once or twice a day.
Possible Serious Side Effects
Serious Infection
Azathioprine can cause a decrease in the number of blood cells in your bone marrow, which may cause serious or life-threatening infections. The risk that the number of blood cells that you have will decrease is highest if you have a genetic (inherited) risk factor. Your doctor may order a test (TPMT) before your treatment to see if you have this risk factor. You and your healthcare provider will monitor for signs and symptoms of infection. Your doctor will order tests before, during, and after your treatment to see if your blood cells are affected by this medication. You may be tested for tuberculosis and hepatitis before beginning treatment.
May increase your risk of developing certain types of cancers, especially skin cancers and lymphoma (cancer that begins in the cells that fight infection).
To reduce your risk of developing skin cancer, take proper precautions such as avoiding prolonged exposure to sunlight, wearing protective clothing, and using sunscreen. Tell your healthcare provider immediately if you notice changes in your skin or any lumps or masses. Call your healthcare provider immediately if you experience night sweats, stomach pain, fever, unexpected weight loss, or easy bruising or bleeding.
Liver injury
If your liver is impaired you may not be able to take azathioprine. Your healthcare provider will order blood tests to test your liver function before and during treatment.
Pancreas Damage
Notify your healthcare provider if you experience severe abdominal pain or fluid build-up in your belly.
Benralizumab is an add-on maintenance treatment for those who are diagnosed with severe eosinophilic asthma and is currently being studied for eosinophilic granulomatosis with polyangiitis (EGPA).
Benralizumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Biologics target certain parts of the immune system to control inflammation. Benralizumab improves breathing by reducing swelling and irritation in the airway. It works by reducing two different types of white blood cells – eosinophils and basophils.
Given by subcutaneous injection.
Possible Serious Side Effects
Serious allergic reaction
Contact your healthcare provider immediately if you have signs of a serious allergic reaction such as swelling of your face, mouth, or tongue; breathing problems, lightheadedness, or hives.
May be used off-label to treat Behçet’s syndrome, cutaneous small-vessel vasculitis (CSVV), normocomplementemic urticarial vasculitis
Colchicine is a medication that reduces inflammation and it is used to treat gout. It has been found to be effective in the treatment of skin-limited vasculitis.
Given orally twice a day. Avoid large amounts of grapefruit or grapefruit juice when taking this medication.
Tell Your Healthcare Provider
- if you have liver or kidney disease
- all medications and supplements you are taking
- if you experience diarrhea, vomiting, or abdominal pain
May be used off-label to treat many forms of vasculitis. Used when the vasculitis is severe, which means it may be life-or organ-threatening. Used to quickly induce remission, after which you will be switched to another treatment.
Cyclophosphamide is a chemotherapy medication. The dose of cyclophosphamide used to treat vasculitis is much lower than the dose used to treat cancer.
Cyclophosphamide can be toxic. The higher the cumulative dose of cyclophosphamide the greater the chance of serious side effects. Healthcare providers carefully weigh the risks and benefits of treatment with cyclophosphamide. Cyclophosphamide is typically only used to treat vasculitis when there is a threat to life or major organ function.
Given orally or by intravenous injection.
Possible Serious Side Effects
Bone Marrow Suppression
Severely suppresses your immune system.
Your medical provider will follow your blood work carefully and will adjust your dose as needed based on your white blood cell count.
Infertility in both men and women
The risk of infertility is dependent mainly on age and cumulative dose. Discuss your risk with your healthcare provider. Discuss egg and sperm banking options as appropriate.
Serious Infection
Your healthcare provider will most likely prescribe Bactrim, a combination of two strong antibiotics, to help protect against a potentially life-threatening infection called Pneumocystis jirovecii pneumonia. Your healthcare provider will also monitor your white blood cell count and will recommend certain precautions based on your infection risk. These precautions might include precautions such as masking and isolating. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Inflammation and bleeding of the lining of the urinary bladder (hemorrhagic cystitis)/bladder cancer
Risk is highest in patients who receive a higher cumulative dose of cyclophosphamide. When using intravenous cyclophosphamide, your healthcare provider may prescribe Mesna, an agent, that may help to protect your bladder. You will also be advised to drink a lot of water unless you are on fluid restrictions.
Increased risk of certain cancers such as leukemia and skin cancer.
Risk is highest in patients who receive a higher cumulative dose. Limiting duration of therapy is important. Discuss with your healthcare provider whether they recommend you receive any additional monitoring for potential cancers.
May be used off-label to treat Behçet’s syndrome. May be occasionally used to treat other forms of vasculitis.
Cyclosporine is a powerful immunosuppressant drug. It belongs to a class of medications called disease-modifying anti-rheumatic drugs (DMARDs).
Cyclosporine may cause growth of extra tissue in your gums. Brush your teeth carefully and see a dentist regularly.
Given orally once or twice a day. Avoid grapefruit or grapefruit juice when taking this medication.
Possible Serious Side Effects
Serious Infection
Cyclosporine suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection.
Birth defects/fetal harm
Use an effective form of birth control. Tell your healthcare provider if you are pregnant or become pregnant while taking cyclosporine.
Kidney injury
Tell your healthcare provider if you have kidney problems. Drink plenty of water. Your healthcare provider will use lab tests to monitor your kidney function.
High blood pressure
Your healthcare provider will closely monitor your blood pressure while you are taking cyclosporine.
May be used off-label to treat cutaneous small-vessel vasculitis (CSVV) and urticarial vasculitis.
Dapsone is a type of antibiotic commonly called a sulfa antibiotic. You should not take dapsone if you have had an allergic reaction to other sulfa drugs. In addition to being an antibiotic, it is useful in the treatment of other conditions by blocking inflammation.
The complete blood cell count (CBC) is regularly monitored as Dapsone can lower the red and white cell counts. Before it is started, a blood test is checked for an enzyme name erythrocytic glucose-6-phophate dehydrogenase (G6PD). People with G6PD deficiency should not take Dapsone as it increases the risk of severe hemolytic anemia.
Given orally.
Possible Serious Side Effects
Liver injury
Tell your healthcare provider if you have ever had liver disease. Monitor for signs or liver injury and inform your healthcare provider if you notice yellowing or the skin or eyes or unusual bruising.
Anemia
Your healthcare provider will monitor blood tests to check for possible side effects such as anemia.
May be used off-label to treat Behçet’s syndrome.
Etanercept is a biologic medication. Biologic medications are complex proteins derived from living organisms. It also belongs to a class of medications called tumor necrosis factor (TNF) inhibitors. TNF is a protein in your body that causes inflammation. TNF inhibitors are drugs that block TNF and reduce inflammation
Given by subcutaneous injection.
Possible Serious Side Effects
Serious Infection
Etanercept suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Rarely, lymphoma (cancer that begins the cells that fight infections) in children and teenagers.
Tell your health care provider if your child develops unexplained weight loss; swollen glands in the neck, underarms, or groin; or easy bruising or bleeding.
Used as the initial remission induction treatment for most forms of vasculitis. Used in lower doses to help maintain remission.
Given orally or by intravenous infusion.
Find out more about glucorticoids (steroids) including what you need to know about adrenal insufficiency, tapering steroids, and how to manage side effects on our Prednisone page.
For some forms of vasculitis, there are FDA approved steroid-sparing treatments. These treatments are typically used in conjunction with steroids to help achieve remission with a lower cumulative does of steroids. They may also be used to help maintain remission.
May be used off-label to treat cutaneous small-vessel vasculitis (CSVV) and urticarial vasculitis.
Hydroxychloroquine belongs to a class of medications called disease-modifying anti-rheumatic drugs (DMARDs). It is believed that hydroxychloroquine alters some functions within the immune system by interfering with communication in the immune system. Hydroxychloroquine is not known to suppress the immune system.
Given orally. Should be taken with food.
Possible Serious Side Effects
Rarely, visual changes or loss of vision
This is more likely to occur if you are over 60 years of age and have been taking high doses of hydroxychloroquine for many years. You should have an annual eye examination while taking hydroxychloroquine. Tell your health care provider if you experience blurred vision, floaters in the eye, seeing flashes of light, or other changes in vision.
Rarely, changes in heart rhythm
This is more likely to occur when hydroxychloroquine is used in combination with certain other medications. Tell your healthcare provider if you have had heart trouble.
Low blood sugar (hypoglycemia)
Hypoglycemia is a rare side effect. You may be asked to monitor your blood sugar. Notify your healthcare provider immediately if you have symptoms of low blood sugar such as confusion, fainting, weakness, nausea, or dizziness.
May be used off-label to treat aortitis, Behçet’s syndrome, and Takayasu arteritis (TAK).
Infliximab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Infliximab blocks the action of tumor necrosis factor (TNF). TNF is a protein in your body that causes inflammation.
Biosimilars are similar to, but not exact copies of, biologic medications. The American Cancer Society’s Understanding Biologic and Biosimilar Drugs is a great resource if you want to learn more about biologics and biosimilars.
Given by intravenous infusion.
Possible Serious Side Effects
Serious Infection
Infliximab suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Hepatitis B virus (HBV) reactivation
If you have had HBV or are a carrier of HBV, taking infliximab could cause the virus to become an active infection again.
Rarely, lymphoma (cancer that begins the cells that fight infections) in children, teenagers, and young adults.
Tell your healthcare provider if your child develops stomach pain; fever; unexplained weight loss; swollen glands in the neck, underarms, or groin; or easy bruising or bleeding.
May be used off-label to treat certain forms of vasculitis.
Leflunomide belongs to a class of medications called disease-modifying anti-rheumatic drugs (DMARDs). Leflunomide works to reduce inflammation by suppressing the immune system. It does this by stopping the body from producing too many of the immune cells that cause inflammation.
Given orally.
Possible Serious Side Effects
Serious Infection
Leflunomide suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Birth defects/fetal harm
Use an effective form of birth control. Men taking leflunomide who wish to have a child should talk to their healthcare provider about how to discontinue leflunomide.
May be used off-label to treat giant cell arteritis (GCA).
Mavrilimumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Mavrilimumab blocks the proinflammatory effects of granulocyte-macrophage colony stimulating factor (GM-CSF).
Given by subcutaneous injection.
Possible Serious Side Effects
Serious Infection
Mavrilimumab suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
May be used off-label to treat many forms of vasculitis.
Methotrexate belongs to a class of medications called disease-modifying anti-rheumatic drugs (DMARDs). Methotrexate works to reduce inflammation by suppressing the immune system.
Given orally or by subcutaneous injection. Your healthcare provider will prescribe a folic acid (folate) supplement to decrease the chance of side effects.
Possible Serious Side Effects
Serious Infection
Methotrexate suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. Your doctor will order tests before, during, and after your treatment to see if your blood cells are affected by this medication. You will need to be tested for tuberculosis and hepatitis before beginning treatment.
Birth defects/fetal harm
Use an effective form of birth control. Methotrexate treatment should be discontinued for at least three months before attempting to become pregnant. Men taking methotrexate who wish to have a child should talk to their healthcare provider.
Liver injury/chronic kidney disease
If your liver and/or kidneys are impaired you may not be able to take methotrexate. Your healthcare provider will order blood tests to test your liver and kidney functions before and during treatment.
May be used off-label to treat many forms of vasculitis.
Mycophenolate mofetil belongs to a class of medications called disease-modifying anti-rheumatic drugs (DMARDs). Mycophenolate mofetil works to reduce inflammation by suppressing the immune system.
Given orally.
Possible Serious Side Effects
Serious Infection
Mycophenolate mofetil suppresses your immune system which makes it harder for your body to fight infection. You and your healthcare provider will monitor for signs and symptoms of infection. Your healthcare provider will also monitor your white blood cell count and will recommend certain precautions based on your infection risk. You may need to be tested for tuberculosis and hepatitis before beginning treatment.
Birth defects/pregnancy loss
Use an effective form of birth control.
Liver injury
Your healthcare provider will order blood tests to test your liver function before and during treatment.
May increase your risk of developing certain types of cancers, especially skin cancers and lymphoma (cancer that begins in the cells that fight infection).
To reduce your risk of developing skin cancer, take proper precautions such as avoiding prolonged exposure to sunlight, wearing protective clothing, and using sunscreen. Tell your healthcare provider immediately if you notice changes in your skin or any lumps or masses.
Call your healthcare provider immediately if you experience night sweats, stomach pain, fever, unexpected weight loss, or easy bruising or bleeding.
May be used off-label to treat urticarial vasculitis.
Omalizumab is a biologic medication. Biologic medications are complex proteins derived from living organisms. Omalizumab targets a protein in your immune system that contributes to allergic reactions.
Given by subcutaneous injection.
Find some great tips on injections
Possible Serious Side Effects
Serious allergic reaction
Contact your healthcare provider immediately if you have signs of a serious allergic reaction such as swelling of your face, mouth, or tongue; breathing problems, lightheadedness, or hives.
Increased chance of parasitic infections
Tell your healthcare provider if you have every been exposed to or infected by parasites such as hookworms, roundworms, threadworms, or whipworms. If you are at high risk of this type of infection, using omalizumab may increase your risk of developing a parasitic infection.
May rarely be used in the treatment of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) when they are severely impacting the kidneys or lungs; anti-GBM disease; cryoglobulinemic vasculitis, when it is severely impacting kidneys; and polyarteritis nodosa (PAN) when it is associated with hepatitis B (Hep B).
Why is it performed? To remove abnormal blood proteins (antibodies) that have built up in the bloodstream.
Where is it performed? In a hospital or infusion center.
How is it performed? An intravenous (iv) tube called a central venous catheter (central line) is placed into a large vein that leads to your heart. During plasma exchange this tube is connected to a large machine. Blood is removed through this central line and enters the machine where it is separated into red cells (cells that carries oxygen to your body), white cells (cells that fight disease), platelets (cell fragments that stop or prevent bleeding), and plasma (liquid content of your blood). The plasma is disposed of and is replaced by donor plasma or by another liquid such as albumin (medicine that can be used to replace plasma). The substitute plasma is recombined with your red cells, white cells, and platelets and given back to you though the central line.
How long does it take? Each session or round of plasma exchange typically lasts a few hours. It is typically performed every 2-3 days for a total of 5-10 sessions.
Possible Side Effects
- Nausea
- Mild tingling of hands, feet, or lips
- Feeling cold
- Worsening anemia in people who already have anemia
Rare Side Effects
- Feeling light-headed or faint
- Low blood pressure
- Muscle cramps
- Low calcium levels
- Central line infection
- Bleeding from the central line
Tell your medical team
- if you notice fever or other signs of infection.
- if you have any redness, pain or bleeding from the central line
- what medications and supplements you are taking
- if you avoid blood products
Vasculitis Medications and Treatments
TAVNEOS® (avacopan) ASN Perspectives_Geetha
Pediatric Medications: What's available for childhood vasculitis
Vasculitis Medications for Children
Current Treatments for GPA and MPA including Avacopan
ACRVF Guidelines Conference: EGPA Breakout
ACR_VF Guidelines: GPA-MPA Breakout
ACRVF Guidelines Conf PAN Breakout
ACRVF Guidelines Conf GCA Breakout
ACR VF Guideline Langford Session 2
Introducing the 2021 ACR/VF Vasculitis Clinical Practice Guidelines Part 1
Introducing the 2021 ACR/VF Vasculitis Clinical Practice Guidelines Part 2: Q&A
Additional Resources
ACR/VF Treatment Guidelines
These guidelines present the first recommendations endorsed by the American College of Rheumatology and the Vasculitis Foundation for the management of some forms of vasculitis.
Prednisone (Glucocorticoids)
Treatment for vasculitis frequently involves taking high doses of glucocorticoids such as prednisone. Glucocorticoids quickly reduce inflammation, protecting vital organs; but they also have a lot of side effects. Learn about possible side effects & how to manage them.
Your Treatment Plan
Developing a treatment plan that is right for you requires careful discussion between you and your healthcare provider, guidance from research studies and treatment guidelines, and the clinical knowledge, experience and expertise of the members of your treatment team.
Immunity, Illness & Infection
When you are being treated for vasculitis, you are usually considered immunocompromised. Learn how to protect yourself from illness and infections, how to time vaccinations, and more.
Vasculitis Treatment & Family Planning
Vasculitis can impact the reproductive health of both men and women. The VPPRN has produced a number of helpful resources on reproductive heath, family planning and vasculitis.
Medication tips from a Pharmacist
Knowing what questions to ask when you begin a new medication is important. We asked a pharmacist, who is also living with vasculitis, to share his most helpful tips.
