Doctors once talked about vasculitis as a fatal condition. But in today’s healthcare world, treatment advances allow for a much different and more optimistic conversation about the disease.
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Various medications can be used to ease the inflammation that vasculitis brings to blood vessels. As that swelling decreases, blood flows better through your system, reducing health risks.
Is this a cure? Not exactly. But with medication, vasculitis can now be managed as a chronic condition — and that’s a significant step forward from the old days.
Learn more about these medications and what they do from rheumatologist Adam Brown, MD.
How do vasculitis medications work?
Let’s start with this reality: Vasculitis is a complex and varied autoimmune disease. There are more than 30 types of vasculitis targeting different parts of the body and different groups of people.
Symptoms and features of the disease run the gamut. Vasculitis could cause nasal congestion or hearing loss, for instance. It could lead to skin lesions, shortness of breath and stiff joints, too. The list goes on and on.
But the common connection is inflammation in blood vessels caused by an overreaction by your immune system. Treatment and medication options to address and reduce that swelling vary case by case.
“Once we determine the type of vasculitis you have, we look at its severity,” explains Dr. Brown. “That helps us gauge how aggressive we need to be with your treatment and what medications to consider.”
Medications typically fall into one of two categories:
- Corticosteroids. Commonly identified by the shortened term “steroids,” these lab-made medications resemble a hormone (cortisol) produced by your adrenal glands. Steroids work to rapidly decrease inflammation and reduce immune system activity.
- Disease-modifying antirheumatic drugs (DMARDs). These medications are referred to as immunosuppressants, given how they change how your immune system reacts. That change helps reduce your body’s inflammation response.
Common medications for vasculitis
Almost all types of vasculitis get treated with a specific class of corticosteroids known as glucocorticoids. For certain types of vasculitis, another medication — typically a DMARD — may be used in tandem.
Here are six of the more commonly used medications. (There are other medications beyond those listed that your doctor may prescribe for vasculitis.)
The potential side effects that are included aren’t intended to be complete. It’s best to review your medication treatment plan carefully with your healthcare provider and pharmacist to understand possible risks specific to your situation.
Prednisone
Prednisone is a corticosteroid (glucocorticoid) that has proven effective in quickly treating inflammation from vasculitis.
Treatment details: Prednisone can be taken as a pill or injected as a shot, typically in the morning. The type and severity of your vasculitis will determine your initial dosage. Over time, dosage levels are usually reduced.
Potential side effects: Increased infection risk is the No. 1 concern with prednisone. Other potential side effects include:
- Increased blood sugar (diabetes).
- Increased blood pressure.
- Loss of bone density (osteoporosis).
- Easy bruising and poor healing.
- Mood swings.
- Insomnia.
Prednisone is also associated with increased appetite and weight gain. People taking prednisone may notice a change in appearance related to the redistribution of normal fat cells in the face and trunk. This usually improves as the dosage is lowered.
Rituximab
Rituximab is a DMARD approved for use in the treatment of two forms of vasculitis — granulomatosis with polyangiitis (GPA or Wegener’s) and microscopic polyangiitis (MPA).
Treatment details: Rituximab is delivered by vein in an infusion center or hospital. The process typically takes four to six hours, though it can run longer. The dosage and frequency of rituximab treatment varies based on several different factors.
Potential side effects: Reactions such as rashes and sores of the skin and mouth sometimes follow a rituximab infusion. There’s also a risk of a rare brain virus infection called progressive multifocal leukoencephalopathy (PML).
Avacopan
This relative newcomer to vasculitis treatment gained approval from the U.S. Food and Drug Administration (FDA) in 2021. Avacopan is used to treat GPA and MPA, two types of anti-neutrophil cytoplasmic antibody (ANCA) vasculitis.
Taking avacopan can help reduce the amount of steroids a patient requires.
Treatment details: Avacopan comes in a tablet and is typically taken twice daily with food. (The dosage may be reduced if you’re taking other medication.) The capsules shouldn’t be crushed, chewed or opened.
Potential side effects: The most common reactions to avacopan during trials included nausea, headache and elevated blood pressure. Liver damage has been reported in people taking avacopan, and monitoring is recommended. There’s also an elevated risk of serious infections.
Cyclophosphamide
When it comes to vasculitis treatment, cyclophosphamide is a tried-and-true DMARD used primarily for severe small- and medium-vessel vasculitis. The medication — known by the brand name Cytoxan® — can be taken orally or intravenously.
Cyclophosphamide is used much less commonly in the treatment of vasculitis than it was 10 years ago.
Treatment details: The medicine can be taken either orally daily or through an IV every two to four weeks. It’s important to hydrate after taking the medication to prevent it from sitting in your bladder.
Potential side effects: Cyclophosphamide can increase your risk of bladder cancer, which is why hydrating is so important to help flush it out of your bladder. The medication can also lower your blood counts, which your provider will probably test every week or two as a precaution.
Nausea and/or vomiting may sometimes occur after the medication is given intravenously. Cyclophosphamide can reduce fertility in women, too. And if you’re pregnant, cyclophosphamide increases the risk of birth defects.
Methotrexate
Methotrexate is a DMARD used to treat vasculitis, as well as other autoimmune conditions.
Treatment details: Methotrexate can be taken once a week as a tablet. Your doctor will base the dosage on your weight and other factors. The medication may be given as an injection just under the skin.
Potential side effects: Methotrexate can be associated with lowering blood counts, irritation of the lungs (pneumonitis) and liver damage. Your provider may order blood tests to monitor your blood counts and liver.
Nausea, vomiting, mouth sores, rash or diarrhea are also possible side effects. Avoid taking methotrexate if you’re pregnant or trying to get pregnant, as it’s been associated with miscarriages and birth defects.
Azathioprine
Doctors mainly use azathioprine as a “maintenance medication” in people with small- or medium-vessel vasculitis after the vasculitis has been managed. It’s taken as a pill.
Treatment details: Prior to beginning treatment, healthcare providers usually perform a blood test to check for a natural body enzyme (thiopurine methyltransferase, or TPMT) that breaks down the medication.
People who don’t make this enzyme can’t take azathioprine. If you make lower amounts of TPMT, the dosages will be smaller.
Those who make enough TPMT are given a dosage based on body weight.
Potential side effects: Nausea, vomiting, abdominal cramping or diarrhea are possible side effects. Taking the medication twice a day instead of all at once, or taking it with meals, often helps minimize these issues.
Azathioprine is also associated with lower blood counts and liver issues, which makes it important to have regular blood tests to watch for problems.
Rarely, allergic reactions to azathioprine require the medication to be stopped.
Long-term outlook
Though outcomes vary case by case, most people eventually see their vasculitis go into remission with medications and treatment, notes Dr. Brown. But relapses can occur. Ongoing monitoring and active communication between you and your healthcare provider are key to keeping the condition at bay.
“We look at vasculitis as a chronic disease,” he says. “While we are unable to cure the condition, we can treat it and put it into remission while carefully watching for any potential side effects.”
