Vasculitis is a complex illness. This spectrum of conditions involving blood vessel inflammation usually has unknown causes — and symptoms can be hard to pin down. The good news? Doctors are making strides in their understanding of these diseases with better drug therapies.
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Rheumatologist Adam Brown, MD, says vasculitis can affect anyone of any age and can damage organs and blood vessels over time. It was once considered fatal. But thanks to medical advances, vasculitis is now manageable as a chronic condition in many cases.
Symptoms are diverse
What makes treating vasculitis a challenge? Here are a few reasons:
- There are several different types of vasculitis.
- Even within a specific disease, the features differ among patients.
- Many organs and/or blood vessels are affected.
- Some forms are mild, others severe.
- It can be secondary to an underlying condition.
- It can be a primary disease with an unknown cause.
There’s no easy way to tell if you have vasculitis because symptoms can vary greatly, Dr. Brown says.
“One type of vasculitis is known as giant cell arteritis, which primarily affects elderly patients,” he says. “It usually presents with new headaches and pain in your jaw while eating. Another type affects your lungs, causing you to have shortness of breath and to cough up blood. There are also forms of vasculitis that can cause big, swollen and painful joints.”
Other symptoms run the gamut. They may include nasal congestion, nose bleeds, mouth ulcers, hearing loss, skin lesions, vision problems, numbness, weakness, cough, shortness of breath, fever and unexplained weight loss.
Treatment options vary among the different types of vasculitis. Doctors treat almost all types with a glucocorticoid medication, such as prednisone. For certain types of vasculitis, another medication may need to be added to prednisone.
“Once we determine the type of vasculitis you have, we look at its severity. That helps us gauge how aggressive we need to be with your treatment and what medications to consider,” Dr. Brown says. “We also have to consider the side effects.”
It’s important to note that medications that treat vasculitis suppress the immune system, and they increase your risk of infections. You want to consider ways to reduce this risk.
Commonly used drugs
Here are some of the more commonly used medications. However, not every one of them apply to all types of vasculitis or to each individual person. In addition, there are other medications that your doctor may prescribe for vasculitis beyond those listed below.
The side effects that are included below aren’t intended to be complete. You should review your treatment plan carefully with your doctor and pharmacist. They can help you understand the risks of their medications — and what can be done to monitor for and prevent side effects.
Prednisone is a glucocorticoid medication (also called a steroid). Glucocorticoids are very valuable in the treatment of vasculitis as they have very broad effects on inflammation and are rapidly acting.
Treatment details: Glucocorticoids are used in almost all forms of vasculitis. They can be given by mouth or by vein. The initial dosage will be determined by your doctor based upon many factors including the type of vasculitis and the severity of the vasculitis. Usually, this is started at a higher dose and then reduced. Prednisone is a relative of a nature body hormone called cortisol which our body delivers in the morning. Because of this, we ideally give prednisone once a day in the morning to replicate what the body does naturally.
Side effects: Increased infection risk is the number one concern with prednisone. Other prominent side effects include increased blood sugar (diabetes), increased blood pressure, loss of bone density (osteoporosis), easy bruising, and poor healing. Prednisone is also associated with increased appetite and weight gain and can result in mood swings and insomnia. People taking prednisone also notice a change in appearance related to redistribution of normal fat cells in the face and trunk which usually improves as the dose is lowered.
2. Rituximab (also called Rituxan)
Doctors have used rituximab to treat rheumatoid arthritis patients, with good results. Since 2011, it has also been approved by the Food and Drug Administration for the treatment of two forms of vasculitis – granulomatosis with polyangiitis (Wegener’s) and microscopic.
Treatment details: Rituximab is given by vein in an infusion center or hospital. The treatment time (infusion) takes four to six hours, or longer in some cases. The dose and frequency of rituximab will be determined by your doctor based on a number of different factors.
Side effects: The main side effects of rituximab are reactions during the infusion, rashes and sores of the skin and mouth. An extremely rare described event is a type of brain virus infection called PML.
3. Cyclophosphamide (also called Cytoxan)
Cyclophosphamide is a tried-and-true older drug, taken orally or intravenously, that doctors also use to treat cancer. It is currently given mainly in the setting of severe small- and medium-vessel vasculitis.
Treatment details: Taken orally, the typical daily dose is 1.0 to 2.0 milligrams per kilogram of body weight than should be taken all at once in morning. Throughout the day, people should drink a large amount of fluid. This helps to flush the medication out of the body in the urine so it is not sitting in the bladder. If your doctor administers the drug intravenously, they’ll determine the dosage based on your height, weight and kidney function levels from what has been used in published studies.
Side effects: Cyclophosphamide has a number of potentially serious side effects that must be understood before this is taken. As it can lower the blood counts, blood tests are performed every one to two weeks. Cyclophosphamide can cause injury to the bladder and potentially bladder cancer. You may experience nausea and/or vomiting which is more common when given by vein. Cyclophosphamide increases the risk of birth defects in pregnant people.
Methotrexate is also used to treat many different autoimmune conditions, including vasculitis. This drug is also used to treat cancer, but the dose used to treat those with cancer is several times higher.
Treatment details: Methotrexate is taken once a week. This drug comes in 2.5-milligram tablets. Your doctor will base the dosage on what has been successfully used in studies, as well as your weight and other factors. The drug can also be given as an injection just under the skin, which some people prefer.
Side effects: You may experience nausea or vomiting. Mouth sores, rash or diarrhea may occur in a small percentage of cases. Methotrexate can be associated with lowering blood counts, irritation of the lungs (called pneumonitis), and liver injury. It is important to avoid drinking alcohol while taking methotrexate. Avoidance of pregnancy is essential as methotrexate can cause miscarriages and birth defects. Your doctor will order blood tests to monitor your blood counts and liver function abnormalities show up in the tests.
5. Azathioprine (also called Imuran)
Doctors mainly use azathioprine as what is referred to as a maintenance medication in people with small- or medium-vessel vasculitis after the vasculitis has been controlled.
Treatment details: Prior to beginning treatment, doctors usually perform a blood test called TPMT which is a natural body enzyme which breaks down the medication. People who do not make this enzyme cannot take azathioprine and people who make lower amounts will need to be treated cautiously with a smaller dose. In those who have a normal TPMT test, the dosage is usually based on your body weight. You may receive a single or twice-daily dose.
Side effects: You may experience nausea and vomiting and, in some cases, abdominal cramping or diarrhea. Taking the drug twice a day instead of all at once, or taking it with meals helps some people avoid these side effects. Azathioprine is associated with lower blood counts and abnormal liver tests, which makes regular blood test monitoring is important. Rarely, people may have an allergic reaction to azathioprine that requires the medication to be stopped.
Stay in touch with your doctor
The effects of vasculitis over time will vary for each person. It depends on the following factors: vasculitis type, severity, response to treatment, side effects of the treatment, and whether or not vasculitis has resulted in any permanent organ damage.
Fortunately for most patients, vasculitis will go into remission following treatment. In remission, no active vasculitis or inflammation is causing injury to tissues or organs. The concern is that for most forms of vasculitis, a relapse or return of vasculitis can occur. Because of this, you need to be continually monitored by a physician experienced in treating vasculitis.
Ongoing monitoring and active communication between you and your doctor is the key to detecting and minimizing any relapses, Dr. Brown says.
“We used to consider some forms of vasculitis as fatal. But now we view this as though it’s a chronic disease,” he says. “While we are unable to cure vasculitis, we can treat it and put it into remission.”