What is Vasculitis?
Vasculitis (vas-kyu-LI-tis) is a general term for a group of uncommon diseases that feature inflammation and damage to the walls of blood vessels. Blood vessels are part of your body’s ciculatory system, also known as the vascular system.
There are three types of blood vessels: arteries that transport oxygen-rich blood away from the heart to the tissues of the body, veins that then return the oxygen-depleted blood from the tissues back to the lungs for oxygen, and tiny networks of capillaries that connect small arteries and veins at the tissue level, completing the circle.
Vasculitis can affect any of these blood vessels, and as a result can disrupt blood flow to tissues throughout the body, such as the lungs, nerves and skin. Vasculitis can be mild or disabling, or even fatal. Patients can have one episode of vasculitis or have repeated episodes over several years. Most types of vasculitis are rare. Vasculitis can occur in people with lupus when the immune system mistakenly attacks blood vessels, or as a result of an infection, a medication, or another disease or condition.
Typically after an injury, the body responds with inflammation. In vasculitis, inflammation is caused by injury to the blood vessels, and may involve warmth, pain, swelling, redness and even loss of function in the affected tissues. This could lead to serious complications depending on which blood vessels, organs or other systems in the body are affected. If the inflammation is severe, the blood vessel may become narrow and restrict blood flow. It is possible that the vessels may even close completely, stretch, weaken and potentially bulge. A bulge in a blood vessel is also called an aneurysm (AN-u-rism). Aneurysms may burst, causing internal bleeding. Each of these disruptions in blood flow have the potential to damage internal organs if severe.
The word vasculitis is derived from the Latin “vasculum”, vessel + “- itis”, inflammation. Another term for vasculitis is angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are inflamed, it is referred to as venulitis.
There are several types of vasculitis diseases and each are defined by the patterns of blood vessel involvement in the body, which organs are affected, and laboratory test abnormalities. Vasculitis affects persons of both sexes and all ages. A few forms of vasculitis affect certain groups of people. For instance, Kawasaki disease occurs only in children. On the other hand, giant cell arteritis occurs only in adults over 50 years old. Read more about specific types of vasculitis below.
This rendering from the National Institute of Health, shows what vasculitis looks like in its various stages.
Figure A: shows a normal artery with normal blood flow. The inset image shows a cross-section of the normal artery.
Figure B: shows an inflamed, narrowed artery with decreased blood flow. The inset image shows a cross-section of the inflamed artery.
Figure C: shows an inflamed, blocked (also called ‘occluded’) artery and scarring on the artery wall. The inset image shows a cross-section of the blocked artery.
Figure D: shows an artery with an aneurysm. The inset image shows a cross-section of the artery with an aneurysm. Back to top
What are the different types of vasculitis?
Examples of vasculitis include:
- Behçet’s disease:Behçets can cause recurrent, painful ulcers (sores) in the mouth, ulcers on the genitals, acne-like skin lesions, and eye inflammation called uveitis. Most common in people ages 20-40.
- Buerger’s disease: In this disease, the blood vessels in the hands and feet tighten or become blocked. This is alsoknown as thromboangiitis (THROM-bo-an-jee-I-tis) obliterans. It is strongly linked to tobacco use and smoking. Symptoms can include pain in the calves or feet when walking or pain in the forearms and hands with activity. Other symptoms include blood clots in the surface veins of the limbs and Raynaud’s phenomenon. In severe cases, ulcers may develop on the fingers and toes, leading to gangrene (death or decay of body tissues).
- Central Nervous System Vasculitis (CNS): This usually occurs as a result of a systemic (affecting the body in an overall way rather than one specific location) vasculitis. Very rarely, vasculitis affects only the brain and/or spinal cord. When it does, the condition is called isolated vasculitis of the central nervous system or primary angiitis (an-jee-I-tis) of the central nervous system. Symptoms may include headaches, fogginess, changes in mental clarity, weakness of the muscles or paralysis.
- Cogan’s syndrome: This can lead to eye inflammation called interstitial keratitis (in-ter-STISH-al ker-ah-TI-tis). The syndrome can also cause hearing changes, including sudden deafness.
- Cryoglobulinemia (KRI-o-GLOB-u-luh-NE-me-uh):Also called “Essential mixed cryoglobulinemia”, can occur alone or with a systemic vasculitis and is most often linked to chronic hepatitis C infection. This refers to abnormal proteins in the blood that form a gel when a blood sample is exposed to temperatures lower than normal body temperature. Symptoms might include: joint aches; weakness; numbness, tingling, and pain in the limbs; kidney inflammation; or a raised, bumpy, reddish-purple skin rash known as palpable purpura (PURR-purr-ah).
- Giant cell arteritis (temporal arteritis): This usually affects the temporal artery, an artery on the side of your head. This condition is also called temporal arteritis. Symptoms of this condition can include headaches, scalp tenderness, jaw pain, blurred vision, double vision, and acute (sudden) vision loss. Giant cell arteritis is the most common form of vasculitis in adults older than 50.
- Henoch-Schönlein purpura (HSP): This type of vasculitis affects the smallest blood vessels (the capillaries) in the skin, joints, intestines, and kidneys and is most common in children but can affect both teens and adults as well. Symptoms often include stomach pain, swollen and achy joints, and signs of kidney damage, such as blood in the urine. Another symptom might show as a bruise-like rash that appears as reddish-purple blotches on the lower legs and buttocks (although it can appear anywhere on the body). Approximately half of all cases in children follow a viral or bacterial upper respiratory infection.
- Hypersensitivity Vasculitis: This affects the skin and is also known as allergic vasculitis, cutaneous (ku-TA-ne-us) vasculitis, or leukocytoclastic (LU-ko-si-TOE-klas-tic) vasculitis. One common symptom is red spots on the skin, usually on the lower legs. An allergic reaction to a medicine or infection often causes this type of vasculitis. Treating the infection or stopping the medication usually clears up the vasculitis. However, some people may need to take anti-inflammatory medicines, such as [corticosteroids], for a short time.
- Kawasaki disease: This is a rare childhood disease in which the walls of the blood vessels throughout the body become inflamed. The disease can affect any blood vessel in the body, including arteries, veins, and capillaries. It is also known as mucocutaneous (myu-ko-kyu-TA-ne-as) lymph node syndrome. This is because the disease is associated with redness of the mucous membranes in the eyes and mouth, redness of the skin, and enlarged lymph nodes. Kawasaki disease can also affect the coronary arteries, which carry oxygen-rich blood to the heart. In a small number of cases where the coronary arteries are affected, children who have Kawasaki disease may also have serious heart problems.
- Microscopic Polyangiitis (POL-e-an-jee-I-tis): This affects small blood vessels, particularly those in the kidneys and lungs. Often, the symptoms aren’t specific, and may begin gradually with muscle aches, weight loss, and fever. Sometimes the symptoms come on suddenly and progress quickly, leading to kidney failure. Coughing up blood may be the first symptom if the lungs are affected. Sometimes it occurs in conjunction with a vasculitis that affects the intestinal tract, the skin, and the nervous system.
- Polymyalgia rheumatic or PMR (POL-e-my-AL-jah RU-ma-ti-kuh): This commonly affects the large joints in the body, such as the shoulders and hips. PMR typically causes stiffness and pain in the muscles of the neck, shoulders, lower back, hips and thighs.
- Takayasu’s arteritis (tock-e-AH-sues):This type of vasculitis affects medium- and large-sized arteries, particularly the aorta and its branches. The condition is sometimes called “aortic arch syndrome”.
- Wegener’s granulomatosis: This is a rare type of vasculitis. It is more common in middle-aged Caucasians. It typically affects the sinuses, nose, and throat; the lungs; and the kidneys as well as other organs. It not only causes inflamed blood vessels, but the affected tissues also develop abnormal lumps called granulomas. If granulomas develop in the lungs, they can destroy lung tissue which can be mistaken for pneumonia or even lung cancer. Symptoms may begin slowly with fever, weight loss, and muscle aches, but sometimes the symptoms come on suddenly and progress rapidly, leading to kidney failure. If the lungs are affected, coughing up blood may be the first symptom.
- Churg-Strauss syndrome: This disorder is very rare and is also known as allergic angiitis and granulomatosis (GRAN-u-lo-ma-TO-sis). Symptoms may vary widely between individuals and can affect many organs, including the lungs, skin, kidneys, nervous system, and heart. They may include asthma, higher than normal levels of white blood cells in the blood and tissues, and abnormal lumps known as granulomas (gran-yu-LO-mas).
- Polyarteritis nodosa (POL-e-ar-ter-I-tis no-DO-suh): This can affect many parts of the body, is rare, and may be linked to hepatitis B or C infections. This disorder often affects the nerves, digestive track, kidneys, and the skin. Symptoms will often include fever, a general feeling of being unwell, weight loss, and muscle and joint aches, including pain in the calf muscles that develops over weeks or months. Anemia, a lace-like rash, bumps under the skin and/or stomach pain after eating might also be sypmtoms.
Vasculitis can also accompany:
- Infections (such as hepatitis B)
- Exposure to chemicals (such as amphetamines and cocaine)
- Cancers (such as lymphomas or multiple myeloma)
- Rheumatic diseases (such as rheumatoid arthritis and systemic lupus erythematosus)
What causes vasculitis?
Although it is unknown what causes most types of vasculitis, immune system abnormality, inflammation of blood vessels, and genetic factors appear to be somewhat important in the cause of the disease. Vasculitis can also be caused by an immune or allergic reaction in the vessel walls. In rare cases, infections of the blood vessel walls can cause vasculitis. In this case, a bacteria or virus infects the blood vessel and then the white blood cells cause damage to the vessel as it destroys the infection.
Vasculitis is thought to be an autoimmune disease, which means the body comes under attack by its own immune system. In vasculitis, the immune system attacks blood vessels. However, some cases of vasculitis are caused by hepatitis C or hepatitis B or other chronic (long-term) infections and some cases are caused by reactions to medicines. Vasculitis can overlap with other rheumatic diseases, mainly including systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. Most patients with vasculitis have none of these diseases but people with these diseases may often also have vasculitis. Back to top
What are the symptoms?
It may be confusing to people who suffer from inflammation due to other autoimmune diseases because typical symptoms of vasculitis often mimic those of lupus, rheumatoid arthritis and Sjogren’s. The signs and symptoms of vasculitis will vary depending on where and what organ tissue’s blood vessels are affected and how severe the inflammation is and what, if any, organs are involved.
Symptoms by organ/area of the body:
- General feeling of being unwell
- Weight loss could potentially be a symptom with some types of vasculitis
- Overall weakness
- Changes in behavior or mood
- Confusion or fogginess
- Usually associated with the small blood vessels in the retina in people with lupus. Symptoms are sometimes nonexistent.
- Visual blurring which comes on suddenly and stays
- Vision loss
- Heaviness in the chest during exertion (made better by rest) – this is unusual in people with lupus
- Chest x-ray findings similar to pneumonia.
- Chronic shortness of breath.
- Blood in the urine
- Red or purple dots, often numerous on the legs
- Larger spots that may look like large bruises
- Hives (uncommon)
- Itchy lumpy rash (uncommon)
- Painful or tender lumps (uncommon)
- Numbness and or weakness
- Inability to move (paralysis)
- Muscle pain
- Dull aching
- A sensation of heat
How is it diagnosed?
To make a diagnosis of vasculitis, your physician will review your medical history, conduct a physical exam and order some specialized lab tests.
The most common tests are:
- A complete blood count (CBC),
- Liver function tests
Other tests that may be ordered are:
Biopsy: This is a surgical removal of a small piece of tissue for inspection under a microscope. It is the best way for your doctor to make a confident and definitive diagnosis as it determines the pattern of blood vessel inflammation in the body. This type of test will be used to detect small-vessel vasculitis of the skin or a kidney or medium-vessel vasculitis of the skin, nerves or brain. Giant-cell arteritis often requires a biopsy to be taken of an artery in the scalp.
Angiography: This is a type of X-ray to look for abnormalities of blood vessels usually used to detect large-vessel vasculitis. This test will show characteristic patterns of inflammation in affected blood vessels.
Exceptions: Behçet’s disease and Kawasaki disease, are usually detected by putting together other clinical findings rather than a biopsy or an angiography. Back to top
How is it treated?
Fortunately, most of the types of vasculitis are treatable if detected early, before any significant organ damage has occurred. Many forms of vasculitis are treated with corticosteroids. Together, you and your doctor will put together the best course of treatment taking into consideration your symptoms, the type of vasculitis with which you have been diagnosed, other medical conditions that you have and any other medications that you may already be taking.
Here are some common medications used to treat vasculitis:
- Glucocorticoids: Glucocorticoids (prednisone, prednisolone or others), often referred to as “steroids,” are strong anti-inflammatory medications, and a very important part of treating most forms of vasculitis. The prescribed dose and length of treatment needed will depend on how severe and how far along the vasculitis is. Steroids can have long-term side effects.
- Immune-suppressing drugs: These types of drugs can have a less seriousside than those of glucocorticoids. This is called “steroid-sparing” treatment. Cyclophosphamide (Cytoxin)is the strongest of these drugs, and doctors may prescribe it when severe vasculitis may endanger the vital organs. For less serious vasculitis, patients may receive Methotrexate, Azathioprine, or any other of these immune-suppressing drugs. Doctors often prescribe these drugs to treat other rheumatic diseases, but they are also useful for vasculitis.
- Newer drugs designed to treat other autoimmune and inflammatory diseases: These types of medications may also help vasculitis. Drugs such as rituximab have been shown by researchers to effectively treat severe cases of certain forms of vasculitis. These include granulomatosis with polyangiitis, microscopic polyangiitis and cryoglobulinemic vasculitis.
- Plasma exchange (“plasmapheresis”): Some patients with the most severe cases of these varying vasculitis diseases may receive intravenous (through the veins) immunoglobulin. This procedure is often called IVIg.
- Surgery: If the vasculitis has caused severe damage, surgery may sometimes be required. A surgery to redirect blood flow around a blockage in a blood vessel, (called vascular bypass grafting) may be the best course of action in these, more severe cases. Depending on where the damage is located, other possible operations are sinus surgery or even a kidney transplant.
The specialists with the most overall knowledge about vasculitis are usually rheumatologists. These are physicians who also treat rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus.
Depending on the severity and other organs that are involved, you may need to see other specialists. Some of these specialists may include:
- Dermatologist (skin doctor)
- Neurologist (nervous system diseases, brain function)
- Ophthalmologist (eye doctor)
- Otorhinolaryngologist (ear nose and throat doctor)
- Nephrologist (kidney doctor) or
- Pulmonologist (lung doctor)
Find a rheumatologist near you!
Living with vasculitis
Vasculitis can be short term or lifelong. If you are diagnosed with vasculitis, the outlook greatly depends on four things:
- What type of vasculitis you have
- Which, if any, organs are affected
- How quickly the disease progresses or worsens
- The severity of your condition at diagnosis
Even with doctors primarily focused on reducing inflammation, there are other symptoms of vasculitis that might cause disruptions in your daily life. Symptoms such as extreme fatigue, muscle and joint pain, or generally feeling unwell can be debilitating and cause emotional stress as you try to continue to navigate through your life with family, work, other commitments, etc. It is important to take care of your mind/body connection and speak to someone (such as a mental health professional) if you think you may be depressed. Being good to yourself will help you better manage the trials and challenges that come with a diagnosis of vasculitis or any other medical condition.
Side effects from medications, especially glucocorticoids (please see our blog on the topic of steroids by clicking here), also can be troubling. Patients taking immunosuppressant medications are also at higher risk for infections. Please speak with your doctor about ways in which you can reduce your risk.
Fortunately, with current treatments, the outcome for patients with vasculitis is often good. Although there is no cure for vasculitis, if treated early, some cases of vasculitis may go into “remission”. This does not mean that the disease is no longer in the body, it simply means that there is currently no disease activity. The symptoms and activity can come back or “flare” at any time. Learning to understand your body and your disease may help you to avoid things that can make your vasculitis worsen or “flare”.
Some patients may never have remission with their vasculitis, however, medications can be very effective at controlling chronic vasculitis symptoms and keeping the disease from progressing so it is very important to follow your physician’s treatment plan.
Even though there is still much that is not known about vasculitis, researchers are continuing their efforts to learn more about the disease, the various types of vasculitis, their causes and seek better treatments for those affected. Back to top
Vasculitis Quick Facts
- Vasculitis is characterized as a group of uncommon diseases which feature inflammation of the blood vessels.
- There are many types of vasculitis, and they may vary greatly in symptoms, severity and duration.
- Symptoms of vasculitis vary greatly and depend upon the organs affected and the severity of the disease.
- Vasculitis can range from mild to life-threatening.
- Permanent damage to organs can be prevented by early detection and treatment.
- The main medications for vasculitis usually include glucocorticoids (prednisone and others)
- Medications that suppress the immune system might also be prescribed which will allow patients to take lower doses of glucocorticoids.
- Diagnosing vasculitis most often includes a biopsy of affected tissue or angiography.
- Treatment is directed toward decreasing inflammation of the blood vessels.
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